TECHNOLOGY EXPLAINER
Blood–Brain Barrier (BBB)
— Crossing an exclusionary barrier by using receptors as carriers
The endothelial cells of the capillaries of the brain are blocked by tight junction. It was practically used as a way to overcome it. transferrin receptors to the cells to pass through the cellsIt is a design. 2021 in Japan, 2026 in the United States,Two different products using the same receptorhas been approved.
- What is a blood brain barrier?
- Wall entities - junctions and people around cells
- There are only two types of roads: physical walls and transport walls
- Passage of transceiver interventive transceiver (RMT)
- "Lowering" affinity, often entering - the reverberation of 2011
- Material Technician Perspective 1: Design for 'Release' without absorption
- Expanding Receptors’ Choices - Select by Protein Subjects
- Two approved products – the same receptor, different structure
- Calculation in this paper: comparing the mass of the carrying side and the size of the carrier
- Material Technician Perspective 2: The contents of freeze-dried products are mostly sugar
- The choice of intrathecal and intrathecal administration
- How to evaluate: From cell models to approved material
- Issues and Undetermined
- Glossary/Reference Materials/Reference Table
Facts= Contents described in the publication material (with source link)
The calculation of this paper= The value calculated based on the specified premise
Undetermined / Future= Items that have not been confirmed by the research stage and plan
In addition to this, it is possible to organize structures and read materials and process designs."Description"is distinguished.
About this articleExplanation of material technology and preparation technologyComment It does not evaluate the effectiveness or safety of individual medicines, compare the product and make it inferior. The structure, composition, and approval of the product are only quoted in the FDA and PMDA publication. The results of animal experiments are not applicable to humans.
1. What is a blood brain barrier?
- How it works:Abbott"Neural signal transmission in the central nervous system (CNS) requires a highly controlled micro-environment."and between blood and CNSThree Interfaces——Blood cerebral cor (BBB), blood cerebro nal cor , Spider membrane cor gate—— organizes the wallFacts
- Where:PublicationsBBB at the level of cerebral microvessels is a place for blood and CNS exchange.Facts
- Why is it a problem with drug discovery?Home"Mono、nal antibody has the possibility of treating central nervous system disease, but the brain is limited by blood brain barrier."FactsHome AbbottEach drug discovery and delivery program targeting or avoiding CNS needs to consider the BBB's speciality.Contact UsFacts
The design that exceeds。B was "dissipate" rather than "st。". HomeAntibodies with reduced affinity to transferrin receptors (TfR) are often migrated into the brainindicatesFacts。 Antibody binding to TfR with high affinity remains in BBBHomeLow variants of affinity were released from BBB to the brain—— Areas where design guidelines are not used to increase the strength of binding(This article explains).
2. Wall entities: junctions and people around cells
The paper is the structure and function of BBB"Physical wall formed by tight junction of endothelial cells"Home"Transportation wall caused by membrane transport and follicle mechanism"I organize it separatelyFacts。
In addition, as a correlation cell"A)site (Glia) foot pro)sion (endfeet), Pericite, Micglia"RolesFacts。
BBB"In short and long term, the adjustment can be disturbed by the disease."Facts。
Abbott et al is another theory,Cerebral microvessels, neurons, and functional neuro units work together.and "The Importance of Endocrinal Cells in This Modular Organization"Facts。
3. There are only two types of roads: physical walls and transport walls
When Abbott organizes, BBB’s “community” is made of two elements:Facts。
| Wall Type | Substrate ( description) | To go beyond it (arrangement by this article) |
|---|---|---|
| Wall | Tight junction of endothelial cellsFormed by | Cell and CellNo gapsHome You only have to pass through the cells |
| Transport Wall | Membrane transport and follicle mechanismFrom | For existing transport systemsContact Us |
Left 2 columnsFacts(Abbott et al 2010 [Reference 1]). Right column is organized by this article.
“I have to go through the cells”——This is the starting point of DDS. and"Mechanism by follicle"The following chapter is actually used to carry medicines.Transceiver TransceiverComment
4. Passage of transceiver interventive transceiver (RMT)
What is transcytosis?Remove from one side of the cell with a follicle, pass through the cell and release it to the other sideContact Us You can use one that connects to the receptor to it. The description is written in the attachment of the approved product.
The Fc (C-terminal) part of the Tibetan alpha-eknm connects to the vertebral domain of the transferin receptor (TfR), and transmits the IDS to the peri-al tissue and the central nervous system by receptor-assisted transcytosis beyond the blood brain barrier.Facts
More“TfR is universally expressed, so it is expected that the interaction between this agent and TfR contributes to the intake of brain and peri、al tissue cells.”See alsoFacts。
This agent is mainly taken into the cells through the mannose-6-phosphate receptor and transferrin receptor 1 (TfR) in the peri-al tissue and organs, and then transported to the lysosome and disassembles the accumulated GAG. In addition, transcytosis via TfR passes through the blood cerebral cor、 (BBB) and decomposes the accumulated GAG after being taken through the peri、al tissue as well as the mannose-6-phosphate receptor and TfR.Facts
BothTransferrin receptorContact Us This receptor for womenProtein (transferin) that carries iron into cellsnot prepared for medicine. Get your luggage on the original logistics path——This is RMT’s way of thinking This article explains.
5. “Re ” the affinity and enter well – the reverse of 2011
After you decide to use RMT, the designer will be asked to:Is it better to strengthen binding to receptors?Home Yu et al 2011 report showed the answer against this intuitionFacts。
"Re、 the affinity of antibody transferin receptors (TfR) indicates that the receptor interceptive transceiver of antibody transceives into the mouse brain beyond the blood cor、 of anti-TfR antibody."Facts
"The anti-TfR antibody that binds with high affinity to TfR remains binding to BBB, while the low affinity anti-TfR antibody mutants are released from BBB to the brain, showing a wide distribution after 24 hours of administration."Facts
Based on this knowledge,Double-specific antibodies that are low affinity to TfR and combine with high affinity to、 BACE1Design and Compared to single-specific anti-BACE1 antibody, it was more concentrated to the mouse brain after one whole body administration, and the decrease in the cerebral amyloid beta was largeReportFacts。
Perspective of materialians 1: Design that 'dissipate' without absorption or adhesive
When handling surface treatment and coupling agent,"How strong the opponent joins?"It tends to be a performance indicator. However, RMT is beyond BBB,The connection must be used only in the "ent " and must be left at the exit(This article) The result of Yu et al shows it.High-affinity antibodies stay in BBB, low-affinity mutants are released into the brainFacts。
This is the world of materialsDesign of transfer film and mold release agentIt is a composition close to. Keep it firmly on the way and leave it in placeHome The same type of failure is applied to the product without being peeled off when only holding force is raised. This article explains.
Design variableOne continuous amount of "combination strength"It is also characteristic. Change the array of antibodies to step-by-step affinity,Find the best——Viewed in this series of “fat nanoparticles” pKa of ionized fat to find the optimal rangeIt is similar to the work and methodology. This article explains.
7. Widen the choice of receptors – choose by protein content
Is there a receptor that can be used other than TfR? ZucheroExplore protein secretion comprehensivelyHow toFacts。
“Transcriptome and Proteome profiling used to explore proteins of mouse endothelial cells (BEC) that can be used to transport antibody beyond BBB”Facts
The result was first negative.“BBB concentrated proteins identified by microarrays have not significantly promoted the intake of antibodies into the brain because of limited proteins.”Facts。
、Isolated Mouse BEC Proteome AnalysisHomeBaysiden, Glut1, CD98hcIdentification of high expression proteins, including: Both antibodies targeting these are significantly concentrated in the brain after the innate administrationHOME CloseAntibodies for CD98hc showed a solid penetration to the brain after systemic administration and demonstrated significant pharmacological responses measured as a decrease in cerebral amyloid betaReportFacts。
The most suggested in this paperThe reason for failure is “because protein is limited”It wasFacts。
No matter how affinity is high, if the number of opponents is not enough, the amount of transportation will not appear. CatalystActive Point DensityContact UsDensityThis is the same story as this article. So how to exploreSearch by FunctionChanged. Measured with protein (proteome) instead of transfer product (microarray)Comment The amount actually thereIn terms of review, we will communicate with the idea of material analysis.
8. Two approved products – the same receptor, different structure
The design using TfR is actually approved.Two products with different structures in Japan and the United Statesbut approved for the same disease (Mucopolysaccharide type II). The following lists only the facts of structure and approval: It does not compare effects and safety.
| 10mg (Japan) | AV)AH | |
|---|---|---|
| Name | Pabinaf Alpha (genetic recombinant) | tividenofusp alfa-eknm |
| Structure | Humanized anti-human transferin receptor mono nal antibody+Human Izlon Acid-2-SulfataseRecombinant Syndrome ProteinHome 2 A-chain (219 amino acids) and 2 B-chains (975 amino acids). B chainAntibody H-chain (γ1) + linker + | fusion of s (IDS) to the N-ter of IgG1fusion protein |
| Weight | About 300,000 | Approx. 110 kDa |
| manufacturing | Chinese hamster ovaries (CHO) cells | Production with CHO cells by recombinant DNA technology |
| BB Pass | Transcytosis via TfRPass to B | Fc TfRCENTRALJoinTransceiver TransceiverBeyond BBB |
| Type | Freeze-dried preparation (white mass or powder), drip static injection | Freeze-dried powder, dissolved and diluted infusion |
| Application | Mucopolysaccharide type II. Approval number 30300AMX00260000 (In the reference of attachment)Approved March 23, 2021 | BLA 761485、March 24, 2026approved (priority review, new active ingredient).Accelerated approval |
IzcargoFacts(PMDA attachment [Reference Material 6]), AV to AHFacts(Reference Material 5 )The range of efficacy/ef s, usage/dose, clinical results, and safety are different from each product, and this article does not compare.
Attachments"This efficacy was approved under rapid approval based on the reduction of heparan sulfate in the cerebro、nal fluid. Co、ation of approval for this efficacy may depend on the clinical validation and description in the validation trial.”Facts。
Trial 1NCT04251026HomeMulti-center, multi-cohort, single-arm, non-controlled test for 47 pediatric patientsHome The average reduction rate from the baseline of heparan sulfate in the cerebro、nal fluid is 91% (95% confidence interval 89% to 92%)CommentFacts。 Being a single-group test without a control group, being evaluated by an alternative indicator (biomarker)Please note.
The structure of the carrier is different to carry the same structure using the same receptor. AntibodiesOtherFc OnlyUseFactsHome How far is the part to be transported is that it is a design choice ( This article explains.
9. Penetration of this paper: comparing the mass of the carrying side and the size of the carrier
Izkargo 10mg (in 1 vial)Facts
- Active : Pabinaf Alpha 12.5 mg(Excessive filling to ensure 10 mg of injectable amount in consideration of loss during preparation)
- Additive: sodium 2.0 2.0 mg/Purified white sugar 187.5 mgSodium Hydrogen Phosphate 5.34 mg / Disodium Phosphate 5.64 mg / Polyoxyethylene (160) Polyoxypropylene 10.8125 mg / Sodium Hydroxide / Hydroxide Dosage
AV)AH (1 Vial)Facts
- Active :tividenofusp alfa-eknm 150 mg
- Additives: Hydrogen Phosphate Di 5 mg/methionine 7.5 mg/s phosphate 7.7 mg/polysolbate 20 mg/s 14.6 mg/Sucrose 300 mg
Convert from the above numbersThe calculation of this paper。
- Additives Total:2.0+187.5+5.34+5.64+0.8125 = 201.29 mg()es sodium hydroxide and hydrochloric acid for dosage)
- Additive / Active :201.29 ÷ 12.5 = Approx. 16 times
- Ratio of purified white sugar to additives:187.5 ÷ 201.29 = about 93%
- Overload Filling Method:12.5 ÷ 10 = 1.25 times(25% overfill)
- AV's AH Additive Total:5+7.5+7.7+3+14.6+300 = 337.8 mg。337.8 ÷ 150 = 2.3 times
- Ratio of sucrose to additives:300 ÷ 337.8 = about 89%
- Ratio of the size of the carrier:300,000 ÷ 110,000 = Approx. 2.7 times(The FDA weight of Izcargo is about 300,000 in PMDA and 110 kDa in AV's AH)
Premises and Limits:The amount per vial, not one dose. Dosage is not included in total. The weight varies depending on the amount of sugar chain. These are intended to see the composition of the formulation from the point of view of the material, not to discuss effects, safety, and dosage.
10. Material Technician Perspective 2: The contents of freeze-dried products are mostly sugar
In Chapter 9 calculations,About 80% of additivesThat isThe calculation of this paperHome IzcargoPurified white sugar 187.5 mgAV AHSucrose 300 mgFacts。
BothFreeze DriedHomeFactsHome Izcargo Attachments"White mass or powder (freeze-dried formulation)"Home AV's AH"White to ash white, freeze dried powder with a cake-like appearance"Contact UsFacts。
CakeAs the expression is shown as it is,Porous SolidsComment SugarStructural materials to make bonesat the same timeProtecting protein from heat and drying stressIt is also (explanation by this article). In this series "mRNA" we treated that the suc、 accounted for the maximum mass as a frozen protector. Even if the form is different, the composition of sugar is the maximum component is common.
Please note the amount of surfactant. IzcargoPolyoxyethylene (160) Polyoxypropylene 0.8125 mgHome AV's AHPolyethylene 20 3 mgFactsHome All1 mg to several mgIt is a small amount. Amount of protein that suppresses degenerative and cohesiveness in the surface (air fluid and container walls)Home No amount for wetness or dispersion——In the same surfactant, if the target is different, the digit is different. This article explains.
In addition, Izcargo attachments are"This agent is overfilled to ensure that the pavinaf alpha (genetic recombinant) 10 mg from 1 vial is injectable in consideration of the loss during preparation"Facts。 25% Overload FillingThe calculation of this paper——If the container is left unchanged or lost, it is installed in the manufacturing side in advanceIt is. In products with the process of changing the solution,The design of containers, plugs and needles directly connects to the yieldIt is the expression of this article.
11. The choice of intrathecal and intrathecal administration
Apart from the ingenuity beyond B,Putting it directly inside the wallThere is also a method called. There are also approved products.The following lists only the facts of the administration route:
| Product | Type | Dosage route (see attached document) | First approval in the United States |
|---|---|---|---|
| SPINRAZA (nusinersen) | Qualified Antis Oligonucleotide (2′-MOE, Phosphoate) | Intramyal administration(intrathecal) | December 23, 2016 |
| QALSODY (tofersen) | 20 base antis) oligonucleotide (5-10-5 MOE gapmer) | Intramyal administration | April 25, 2023 |
| BRINEURA (cerliponase alfa) | Recombinant human tripeptide zir peptidease-1 (544 amino acids, average 59 kDa) produced by CHO cells | Brain(intraventricular) | April 27, 2017 |
AllFacts(Reference Material 8-10)This article does not handle efficacy, effect, usage, dose, clinical results, and safety.The nucleic acid drug itself is treated with this series "nucleic acid medicine".
12. How to evaluate: From cell models to approved material
How to measure whether to exceed。B? We will organize it within the scope that can be confirmed from the publication material.
| Stage | Used | What to do |
|---|---|---|
| Cell Model | Based on first-generation or cata phic cell strainsCulture Model | Helms‘Developed in vitro the transport of drug to the brain and to facilitate research on the secretion and pathophysiology of endothelial cells.'HOMEVerification (validation) in light of establishedBBB propertiesI have an overview of the existing model. About Us"The ideal BBB cell culture model is not yet developed"Comment |
| Search | Brain CellsTranscriptum Proteome Analysis | ZucheroProteome analysis of iso mouse endocrine cellsIdentify high expression protein fromConcentration of antibodies to them in vivo |
| Check with animals | MouseIntraceral distribution after systemic administrationdisease model animals | HomeIntraceral distribution after 24 hoursComment Izcargo AttachmentsMucopolysaccharide type II model mouseintravenous administration toReduction of GAG in the brain, serum, heart, liver, and spleenindicates that |
| Human indicators | Biomarkers in CSF | AV’s AHReduction of heparan sulfate in CSFClose Attachments'In the currently available bioanalytical method, it is possible to directly quantify the concentration of human CSF or urine intact or DS'HOMEEstimates relative concentrations that are also used for the evaluation of specific diaccharides after onContact Us More“Depending on the difference in bioanaly、 methods, we cannot compare the meaning of other clinical trials.”Contact Us |
AllFacts(Helms et al 2016 [Reference Material 4], Zuchero et al 2016 [Reference Material 14], Yu et al 2011 [Reference Material 3], PMDA attachment [Reference Material 6], FDA attachment [Reference Material 5]).
AV’s AH'HS to directly quantify the concentration of intact or DS'Don’t miss out on material analysisFacts。
What is measured?Specific disaccharides after cutting with srelative concentration from there. More“Depending on the difference in bioanaly、 methods, we cannot compare the meaning of other clinical trials.”nailed withFacts。
If the analysis method is different, the number can not be compared with the same name indicator. The same applies to the measurement of molecular weight of polymers and the identification of ceramics (in this article). The limit is expressly written by the approval document.This is a reference for supplying materials to the regulatory industry.
13. Issues and Undetermined
(1) Quick approval is "verified"
AV’s AHQuick approval based on reduction of heparan sulfate in CSFand the attachment itself "Co、ation of approval for this efficacy may depend on the clinical validation and description in the validation trial."Contact UsFactsUndetermined / Future。
(2) Approval products using non-TfR receptors cannot be confirmed
ZucheroCD98hc, baysiden, Glut1is a mouse report.Facts。 The primary information indicating that the product for human use has been approved was not confirmed within the scope of the survey.Undetermined / Future。
(3) BBB pass by nanoparticles is not handled in this article
There are many studies that use liposome and lipid nanoparticles to send to the brain. In the scope of this article, it is not stated because it is not possible to confirm the approved nanoparticle formulation specified in the attached document that delivers to the brain beyond BBB. Nanoparticles are handled by this series "DDS and targeting" and "lipid nanoparticles".
- The physical BBB is made of physical walls by tight junctions and transport walls by carriers and follicles.Facts
- Practical pathways are receptor interchangeable transcytosis.Both approved products use transferin receptorsFacts
- The affinity to the receptor is lowered to the brainThere is a mouse reportFacts
- Candidates of receptors can now be found in the expression amount.If the amount is not sufficient, the transport amount will not appearFacts
- In the same receptor and the same , there are two types of carriers: whole antibody and only Fc.The ratio of weight is about 2.7 timesThe calculation of this paper
- Additives for freeze-dried products are approximately 80% sugarThe calculation of this paper
14. Glossary
- Blood Brain Gate (BBB)
- The barrier between the blood and the brain, created by the capillary endothelium. Strictly limits the entry and exit of substances.
- tight junction
- A structure that adheres to neighboring cells without gaps. physical wall entity.
- Endothelial cells
- cells that cover the inside of the blood . This is the main role of BBB in the brain.
- A receptor site
- A type of astrocyte cell in the brain. A proliferation swells the blood.
- Cells exposed to the outside of capillaries. BBB
- Neuro Unit
- How brain micro and a site neurons work together.
- Transcytosis
- Transport from one side of the cell to the other side.
- RMT
- Transceiver intervention. How to connect to receptors to pass through cells.
- Transferrin receptor (TfR)
- Receptor that takes protein that carries iron. Used as a vehicle beyond。B.
- affinity
- Binding strength. If you go beyond B, you can not leave the brain too strong.
- Double specific antibodies
- Antibodies that can be combined to two opponents. Used for one-way work design.
- Fc
- A fragment of the antibody’s working area. Interaction with receptors and residue in the body.
- Fusion Protein
- It is made as a single protein by connecting different proteins at a gene level.
- Intramyal administration
- How to directly take the spinal cord in a space. No need to cross the。B.
- Brain
- How to administer directly to the brain room.
- Quick approval
- US approval system based on alternative indicators. Prerequisite for verification tests.
- Freeze Dried
- Freezing and sublimated solid preparation. melt when used. Sugar is responsible for skeletal and protection.
15. Reference Materials
- Abbott, N.J., Patabendige, A.A.K., Dolman, D.E.M., Yusof, S.R., Begley, D.J.「Structure and function of the blood-brain barrier」Neurobiology of Disease 37(1), 13–25 (2010). doi:10.1016/j.nbd.2009.07.030 — pubmed.ncbi.nlm.nih.gov
- Abbott, N.J., Rönnbäck, L., Hansson, E.「Astrocyte-endothelial interactions at the blood-brain barrier」Nature Reviews Neuroscience 7(1), 41–53 (2006). doi:10.1038/nrn1824 — pubmed.ncbi.nlm.nih.gov
- Yu, Y.J.「Boosting brain uptake of a therapeutic antibody by reducing its affinity for a transcytosis target」Science Translational Medicine 3(84), 84ra44 (2011). doi:10.1126/scitranslmed.3002230 — pubmed.ncbi.nlm.nih.gov
- Helms, H.C.「In vitro models of the blood-brain barrier: An overview of commonly used brain endothelial cell culture models and guidelines for their use」Journal of Cerebral Blood Flow & Metabolism 36(5), 862–890 (2016). doi:10.1177/0271678X16630991 — pubmed.ncbi.nlm.nih.gov
- FDA/NLM DailyMed"AV AH (tividenofusp alfa-eknm)" — dailymed.nlm.nih.gov
- PMDA"10mg attachment for Izkargo drip static injection" (JCR Pharma, approval number 30300AMX00260000) (PDF) — pmda.go.jp
- FDA Drugs@FDA"BLA 761485 AV」AH" Approval History — accessdata.fda.gov
- FDA/NLM DailyMedSPINRAZA (nusinersen) dailymed.nlm.nih.gov
- FDA/NLM DailyMedQALSODY (tofersen) Attachment — dailymed.nlm.nih.gov
- FDA/NLM DailyMed"BRINEURA (cerliponase alfa)" — dailymed.nlm.nih.gov
- FDA Drugs@FDANDA 209531 SPINRAZA Approval History — accessdata.fda.gov
- FDA Drugs@FDANDA 215887 QALSODY Approval History — accessdata.fda.gov
- FDA Drugs@FDABLA 761052 BRINEURA Approval History — accessdata.fda.gov
- Zuchero, Y.J.Y.「Discovery of Novel Blood-Brain Barrier Targets to Enhance Brain Uptake of Therapeutic Antibodies」Neuron 89(1), 70–82 (2016). doi:10.1016/j.neuron.2015.11.024 — pubmed.ncbi.nlm.nih.gov
- ClinicalTrials.gov「NCT04251026:A Study of Tividenofusp Alfa (DNL310) in Pediatric Participants With Hunter Syndrome」 — clinicaltrials.gov
16. Response Table of Claim and Sources (Audit)
| Content | Home | |
|---|---|---|
| Neural signal transmission in CNS requires a highly controlled microenvironment. There are three interfaces between blood and CNS: blood-cerebral cor, blood-cerebro fluid cor, and blood-gumo membrane cor. BBB of the endothelial level of cerebral micro vessels is a place for blood and CNS exchange. The distinction between physical barriers by tight junctions of endothelial cells and transport barriers by membrane transport and follicle mechanisms. The involvement of site-specific processes, perisites, and microglia. BBB function is disturbed in the condition of short-term and long-term regulation. The drug discovery and delivery program targeting and avoiding CNS must consider the variability. | Abbott et al (2010)Source 1 https://pubmed.ncbi.nlm.nih.gov/19664713/ | Facts |
| How brain micro and site neurons work together to form a functional 'neural unit' and the importance of cerebral cells in its modularization. | Abbott et al (2006)Source 2 https://pubmed.ncbi.nlm.nih.gov/16371949/ | Facts |
| Mono nal antibody has the potential to treat CNS disease, but the brain is limited by BBB. By lowering the affinity of TfR, the receptor intersection of anti-TfR antibody has increased, and the concentration of treatment meaning is reached within the mouse brain. High-affinity anti-TfR antibody binds to BBB, low-affinity mutants are released from BBB to the brain and indicate a wide distribution after 24 hours of administration. TfR designed high-affinity double-specific antibodies for low-affinity and BACE1, and combined with single-specific anti-BACE1 antibodies once a whole body administration, the reduction of Aβ in the brain was large. | Yu et al (2011)Source 3 https://pubmed.ncbi.nlm.nih.gov/21613623/ | Facts |
| The culture model based on the primary or degenerative brain cell strain has been developed to facilitate the study of in vitro models of drug transport to the brain and the uterine and pathological physiology of endothelial cells. The view of the existing model from the perspective of verifying the characteristics of the established BBB. The ideal BBB cell culture model has not yet been developed. | Helms et al (2016)Source 4 https://pubmed.ncbi.nlm.nih.gov/26868179/ | Facts |
| Tividenofusp alfa-eknm is a fusion protein that combines IDS to the N-terminus of the Fc region of IgG1. It is produced by CHO cells using recombinant DNA technology, and the molecular weight is approximately 110 kDa. One vial contains 150 mg of active ingredient, 5 mg of hydrogen phosphate, 5 mg of methionine, 7.5 mg of sodium phosphate, 7.7 mg of polyethylene glycol, 20 mg of polypropylene glycol, and 14.6 mg of sodium. It is a freeze-dried powder with the appearance of a white to ash-white cake. The Fc region binds to the vertical domain of TfR and delivers IDS to perivascular tissue and the CNS beyond the BBB via receptor-mediated transcytosis; TfR is expressed universally, contributing to uptake by brain and perivascular cells. Rapid approval based on efficacy derived from the reduction of heparan sulfate in CSF, and the rationale for approval may depend on clinical validation and description in the validation test. Trial 1 (NCT04251026) was a Phase 1/2 multi-center, multi-cohort, single-group, non-blind test for 47 patients, and 44 patients measured at 24 weeks had an average reduction of 91% (95% CI 89% to 92%) from the baseline of CSF HS. It is not possible to directly quantify the concentration of non-sulfated HS/DS, estimate the relative concentration of disaccharides, or compare the significance of other clinical trials due to differences in biomarkers. | FDA AV AHSource 5 https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=014d92c1-b643-4680-8ca6-f0b3307da915 | Facts |
| Pabinaf, Alpha is a gene-recombinant fusion carbohydrate protein consisting of a humanized anti-human transferrin receptor monoclonal antibody and human isozyme-2-fatty acid esterase (approximately 300,000 CHO weight), produced by CHO cells. The protein consists of a chain of 219 amino acids, with 2 A chains and 2 B chains of 975 amino acids. The B chains consist of antibody H chains (γ1), linkers, and other components. The protein is taken through the ion-independent mannose-6-phosphate receptor and transferrin receptor (TfR), and then transcytosed via TfR to pass through the BBB and enter the brain cells. A decrease in GAG in CSF, brain, serum, heart, liver, and spleen was observed by intravenous administration of a mucopolysaccharide type II model mouse. The composition of mucopolysaccharide type II in one vial (active ingredient 12.5 mg, sodium 2.0 mg, purified white sugar 187.5 mg, sodium phosphate hydrate 5.34 mg, sodium phosphate hydrate 5.34 mg, polyoxyethylene (160) polyoxypropylene 0.8125 mg, sodium hydroxide and hydrochloric acid dosage). must be overloaded to ensure 10 mg as an injectable amount. The sexual form is white mass or powder (freeze-dried formulation). The approval number is 30300AMX00260000, and the quotation is not approved on March 23, 2021. | PMDA 10mg Attachment for Izcargo Drop Quiet InjectionSource 6 https://www.pmda.go.jp/PmdaSearch/iyakuDetail/ResultDataSetPDF/530210_3959424D1023_1_03 | Facts |
| AV AVAH (BLA 761485, Denali The)eutics) has been approved on March 24, 2026 and must be a priority review and a new active ingredient (Type 1). | Drugs@FDASource 7 https://www.accessdata.fda.gov/scripts/cder/daf/index.cfm?event=overview.process&ApplNo=761485 | Facts |
| nusinersen is an antis oligonucleotide with 2-'-O-(2-methoxyethyl) modifier and phosphatioate binding, and is administered intrathecal | FDA SPINRAZASource 8 https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=dd70cd5f-b0fc-4ba4-a5ea-89a34778bd94 | Facts |
| tofersen is an anti-s oligonucleotide of 20 bases 5-0-5 MOE gapmer type and is administered intrathecal | FDA QALSODYSource 9 https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=81356b45-1cb7-4eef-88ea-e44cc18b47c5 | Facts |
| cerliponase alfa is a recombinant human tripeptide zir peptidease-1 (544 amino acids, average 59 kDa) produced by CHO cells and is administered in the brain | FDA BRINEURASource10 https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=485ab3c5-0359-4878-872f-2fe1e7df4047 | Facts |
| SPINRAZA (NDA 209531) was approved on December 23, 2016 | Drugs@FDASource11 https://www.accessdata.fda.gov/scripts/cder/daf/index.cfm?event=overview.process&ApplNo=209531 | Facts |
| QALSODY (NDA 215887) has been approved by priority screening on April 25, 2023 | Drugs@FDASource12 https://www.accessdata.fda.gov/scripts/cder/daf/index.cfm?event=overview.process&ApplNo=215887 | Facts |
| BRINEURA (BLA 761052) has been approved for priority screening on April 27, 2017 | Drugs@FDAReference13 https://www.accessdata.fda.gov/scripts/cder/daf/index.cfm?event=overview.process&ApplNo=761052 | Facts |
| The profiling of the transcriptome and proteome explores proteins of mouse endothelial cells that can be used to transport antibodies beyond the BBB. Since the presence of proteins is limited, antibodies for BBB concentrated proteins identified by targets and microarrays listed in the literature have not significantly promoted the intake into the brain. Identification of high expression proteins, including bacilidine, Glut1, and CD98hc, from the proteome analysis of the isozyme-2 mouse endothelial cells, and these antibodies were significantly concentrated in the brain after administration. An antibody against CD98hc showed a significant brainulation to the brain after systemic administration and showed significant pharmacological responses measured as a decrease in Aβ in the brain. | Zuchero 2016Reference14 https://pubmed.ncbi.nlm.nih.gov/26687840/ | Facts |
| A Study of Tividenofusp Alfa (DNL310) in Pediatric Participants Withrome Syndrome | ClinicalTrials.govSource15 https://clinicaltrials.gov/study/NCT04251026 | Facts |
| Additives total of 201.29 mg, additive / active ingredient about 16 times, purified white sugar about 93% of additives, overfill 1.25 times (25%). AV。AH additives total 337.8 mg, additives / active ingredients about 2.3 times, about 89% of additives with suc。. Ratio of weight of carrier 300,000 ÷ 110,000 = 2.7 times | Calculation and deconstruction of the attached document. Dosages are not included in total. The amount per vial varies depending on the amount of sugar chain. Effects, safety, and dosage. | The calculation of this paper |
| Fast approval of AV。AH depends on the result of the validation test. Approval status of human products using CD98hc, bacilidine, and Glut1 | The attachment itself is dependent on the validation test. The primary information indicating product approval using CD98hc was not confirmed at the time of the investigation (September 2026). | Undetermined / Future |
| Approved nanoparticle preparations specified in the attached document to deliver to the brain beyond the BBB. | I can't confirm the scope of the survey. | |
| ["cells that you only have to pass through. RMT was expressed as “to put luggage on the original logistics path”. The design of affinity has been found in transfer films and mold release agents. Read that the receptor search has switched from "Search by Function" to "Search by Expression" and it has become the active point and sensual point. The sugar is positioned as a structural and protective material for freeze-dried products. Read that the amount of surfactant is different depending on the purpose. I read that the design of containers, plugs and needles is directly connected to the yield from overload filling. If the analysis method is different, it cannot be compared with the same indicator. 3 ways to face the challenge"] | and commentary of this article based on the publication content. It is not the opinion of authors and regulatory authorities of each paper | |
| Comparison of the effectiveness and effect of each product, usage and dosage, clinical results, safety evaluation and product-to-product | This article is an explanation of material technology and preparation technology, and does not evaluate and compare the effects and safety of the treatment. Only the contents of the structure, composition, and approval described in the publication material of the regulatory authorities were quoted for approved products. Chapter 8: The number of heparan sulfate in CSF is described in the FDA attachment as an alternative indicator of a single-group trial without a control group | |
| Fig.1, Fig.3, Fig.5, is a diagram for explanation, not the actual tissue, cell, and。. Hero image and figure 2 are AI generation images | Notes by this article |
Last Updated: September 26, 2026/Source is limited to primary information (FDA approved attachments, Drugs@FDA, PMDA attachments, ClinicalTrials.gov, reader review papers). They are distinguished from the facts and are referred to as "explanation" because they contain references to materials and preparation design. The relationship between affinity and brain migration, the search of receptors, and the results of the disease model are both animal experiments and are not useful in humans. AV。AH’s efficacy is a quick approval based on alternative indicators, and the co。ation of approval may depend on the validation test and is described in the attachment. The approved products using non-TfR receptors and approved nanoparticle preparations that specify the BBB pass cannot be confirmed by the public primary information. This article explains materials technology and preparation technology.It does not evaluate the effectiveness and safety of the treatment or compare the product, and it is not a medical advice. All diagrams are illustrations for explanation. Figure 1, Figure 3 to Figure 5 is a vector drawing, and Figure 2 is an AI-generated image.It is not a picture of the actual tissue, cells, s and products.