phenylbutanoic acid 🐶 Veterinary Use | Indications/Contra | FAERs-F | FAERs-M | Orange Bk | BioActivity |
| Stem definition | Drug id | CAS RN |
|---|---|---|
| 24 | 1821-12-1 |
Description:
| Molecule | Description |
|---|---|
|
Synonyms:
|
Approved in combination with taurursodiol, as relyvrio, for the treatment of amyotrophic lateral sclerosis (ALS) in adults.The mechanism by which relyvrio exerts its therapeutic effects in patients with ALS is unknown.
Legend: OFP - off patent OFM - off market ONP - on patent |
Drug dosage:
| Dose | Unit | Route |
|---|---|---|
| 20 | g | O |
ADMET properties:
| Property | Value | Reference |
|---|---|---|
| BA (Bioavailability) | 78 % | Kim MT, Sedykh A, Chakravarti SK, Saiakhov RD, Zhu H |
Approvals:
| Date | Agency | Company | Orphan |
|---|---|---|---|
| April 30, 1996 | FDA | HYPERION THERAPS INC |
FDA Adverse Event Reporting System (Female)
| MedDRA adverse event term | Likelihood ratio | Likelihood ratio threshold | Patients taking drug having adverse event | Patients taking drug not having adverse event | Patients not taking drug having adverse event | Patients not taking drug not having adverse event |
|---|---|---|---|---|---|---|
| Hyperammonaemia | 116.85 | 50.12 | 18 | 113 | 5214 | 63483677 |
FDA Adverse Event Reporting System (Male)
| MedDRA adverse event term | Likelihood ratio | Likelihood ratio threshold | Patients taking drug having adverse event | Patients taking drug not having adverse event | Patients not taking drug having adverse event | Patients not taking drug not having adverse event |
|---|---|---|---|---|---|---|
| Hyperammonaemic crisis | 66.09 | 47.33 | 7 | 110 | 56 | 34956758 |
FDA Adverse Event Reporting System (Geriatric)
| MedDRA adverse event term | Likelihood ratio | Likelihood ratio threshold | Patients taking drug having adverse event | Patients taking drug not having adverse event | Patients not taking drug having adverse event | Patients not taking drug not having adverse event |
|---|---|---|---|---|---|---|
| Hyperammonaemia | 113.92 | 53.76 | 20 | 168 | 11073 | 79733127 |
| Hyperammonaemic crisis | 85.56 | 53.76 | 9 | 179 | 98 | 79744102 |
FDA Adverse Event Reporting System (Pediatric)
None
Pharmacologic Action:
| Source | Code | Description |
|---|---|---|
| ATC | A16AX03 | ALIMENTARY TRACT AND METABOLISM OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS Various alimentary tract and metabolism products |
| MeSH PA | D000970 | Antineoplastic Agents |
| CHEBI has role | CHEBI:35610 | antineoplastic agents |
| CHEBI has role | CHEBI:50266 | Prodrugs |
| CHEBI has role | CHEBI:61115 | histone deacetylase inhibitors |
| CHEBI has role | CHEBI:68495 | Type I cell-death inducers |
| CHEBI has role | CHEBI:63726 | neuroprotective agents |
| CHEBI has role | CHEBI:71031 | orphan drugs |
| CHEBI has role | CHEBI:176497 | geroprotectors |
Drug Use | Suggest Off label Use Form| |View source of the data|
| Disease | Relation | SNOMED_ID | DOID |
|---|---|---|---|
| Disorder of the urea cycle metabolism | indication | 36444000 | DOID:9267 |
| Congenital hyperammonemia, type I | indication | 62522004 | |
| Ornithine Carbamyltransferase Deficiency | indication | 80908008 | DOID:9271 |
| Amyotrophic lateral sclerosis | indication | 86044005 | DOID:332 |
| Citrullinemia | indication | 398680004 | DOID:9273 |
| Deficiency of carbamylphosphate synthetase (CPS) | indication | 765329008 | DOID:9280 |
| Hyperammonemia | off-label use | 9360008 | |
| Arginase deficiency | off-label use | 23501004 | DOID:9278 |
| Arginosuccinate Lyase Deficiency | off-label use | 41013004 | DOID:14755 |
| Isovaleryl-CoA dehydrogenase deficiency | off-label use | 87827003 | DOID:14753 |
| Kidney disease | contraindication | 90708001 | DOID:557 |
| Disease of liver | contraindication | 235856003 | DOID:409 |
🐶 Veterinary Drug Use
None
🐶 Veterinary products
None
Acid dissociation constants calculated using MoKa v3.0.0
| Dissociation level | Dissociation constant | Type (acidic/basic) |
|---|---|---|
| pKa1 | 4.65 | acidic |
Orange Book patent data (new drug applications)
| Formulation strength | Trade name | Applicant | Application number | Approval date | Type | Dose form | Route | Patent number | Patent expiration date | Patent use |
|---|---|---|---|---|---|---|---|---|---|---|
| 3GM/PACKET; 1GM/PACKET | RELYVRIO | AMYLYX | N216660 | Sept. 29, 2022 | RX | FOR SUSPENSION | ORAL | 10251896 | Dec. 24, 2033 | TREATMENT OF AMYOTROPHIC LATERAL SCLEROSIS (ALS) IN ADULTS |
| 3GM/PACKET; 1GM/PACKET | RELYVRIO | AMYLYX | N216660 | Sept. 29, 2022 | RX | FOR SUSPENSION | ORAL | 10857162 | Dec. 24, 2033 | TREATMENT OF AMYOTROPHIC LATERAL SCLEROSIS (ALS) IN ADULTS |
| 3GM/PACKET; 1GM/PACKET | RELYVRIO | AMYLYX | N216660 | Sept. 29, 2022 | RX | FOR SUSPENSION | ORAL | 9872865 | Dec. 24, 2033 | TREATMENT OF AMYOTROPHIC LATERAL SCLEROSIS (ALS) IN ADULTS |
| 2GM/PACKET | OLPRUVA | ACER | N214860 | Dec. 22, 2022 | RX | FOR SUSPENSION | ORAL | 11202767 | Oct. 17, 2036 | TREATMENT OF A UREA CYCLE DISORDER INVOLVING DEFICIENCIES OF CARBAMYLPHOSPHATE SYNTHETASE, ORNITHINE TRANSCARBAMYLASE, OR ARGININOSUCCINIC ACID SYNTHETASE |
| 3GM/PACKET | OLPRUVA | ACER | N214860 | Dec. 22, 2022 | RX | FOR SUSPENSION | ORAL | 11202767 | Oct. 17, 2036 | TREATMENT OF A UREA CYCLE DISORDER INVOLVING DEFICIENCIES OF CARBAMYLPHOSPHATE SYNTHETASE, ORNITHINE TRANSCARBAMYLASE, OR ARGININOSUCCINIC ACID SYNTHETASE |
| 4GM/PACKET | OLPRUVA | ACER | N214860 | Dec. 22, 2022 | RX | FOR SUSPENSION | ORAL | 11202767 | Oct. 17, 2036 | TREATMENT OF A UREA CYCLE DISORDER INVOLVING DEFICIENCIES OF CARBAMYLPHOSPHATE SYNTHETASE, ORNITHINE TRANSCARBAMYLASE, OR ARGININOSUCCINIC ACID SYNTHETASE |
| 5GM/PACKET | OLPRUVA | ACER | N214860 | Dec. 22, 2022 | RX | FOR SUSPENSION | ORAL | 11202767 | Oct. 17, 2036 | TREATMENT OF A UREA CYCLE DISORDER INVOLVING DEFICIENCIES OF CARBAMYLPHOSPHATE SYNTHETASE, ORNITHINE TRANSCARBAMYLASE, OR ARGININOSUCCINIC ACID SYNTHETASE |
| 6.67GM/PACKET | OLPRUVA | ACER | N214860 | Dec. 22, 2022 | RX | FOR SUSPENSION | ORAL | 11202767 | Oct. 17, 2036 | TREATMENT OF A UREA CYCLE DISORDER INVOLVING DEFICIENCIES OF CARBAMYLPHOSPHATE SYNTHETASE, ORNITHINE TRANSCARBAMYLASE, OR ARGININOSUCCINIC ACID SYNTHETASE |
| 6GM/PACKET | OLPRUVA | ACER | N214860 | Dec. 22, 2022 | RX | FOR SUSPENSION | ORAL | 11202767 | Oct. 17, 2036 | TREATMENT OF A UREA CYCLE DISORDER INVOLVING DEFICIENCIES OF CARBAMYLPHOSPHATE SYNTHETASE, ORNITHINE TRANSCARBAMYLASE, OR ARGININOSUCCINIC ACID SYNTHETASE |
Orange Book exclusivity data (new drug applications)
| Formulation strength | Trade name | Applicant | Application number | Approval date | Type | Dose form | Route | Exclusivity date | Description |
|---|---|---|---|---|---|---|---|---|---|
| 3GM/PACKET; 1GM/PACKET | RELYVRIO | AMYLYX | N216660 | Sept. 29, 2022 | RX | FOR SUSPENSION | ORAL | Sept. 29, 2027 | NEW CHEMICAL ENTITY |
| 3GM/PACKET; 1GM/PACKET | RELYVRIO | AMYLYX | N216660 | Sept. 29, 2022 | RX | FOR SUSPENSION | ORAL | Sept. 29, 2029 | TREATMENT OF AMYOTROPHIC LATERAL SCLEROSIS (ALS) IN ADULTS |
Bioactivity Summary:
| Target | Class | Pharos | UniProt | Action | Type | Activity value (-log[M]) | Mechanism action | Bioact source | MoA source |
|---|---|---|---|---|---|---|---|---|---|
| Aldose reductase | Enzyme | IC50 | 4.02 | CHEMBL | |||||
| Histone deacetylase 2 | Enzyme | IC50 | 4.19 | CHEMBL | |||||
| Histone deacetylase 1 | Enzyme | IC50 | 4.19 | CHEMBL | |||||
| Histone deacetylase 8 | Enzyme | IC50 | 4.03 | CHEMBL |
External reference:
| ID | Source |
|---|---|
| D05868 | KEGG_DRUG |
| 4346-18-3 | SECONDARY_CAS_RN |
| 1716-12-7 | SECONDARY_CAS_RN |
| 1545685 | RXNORM |
| CHEBI:75316 | CHEBI |
| CLT | PDB_CHEM_ID |
| CHEMBL1469 | ChEMBL_ID |
| 4775 | PUBCHEM_CID |
| CHEMBL1746 | ChEMBL_ID |
| CHEMBL1044 | ChEMBL_ID |
| C075773 | MESH_SUPPLEMENTAL_RECORD_UI |
| C000723627 | MESH_SUPPLEMENTAL_RECORD_UI |
| 8480 | IUPHAR_LIGAND_ID |
| DB06819 | DRUGBANK_ID |
| 387058003 | SNOMEDCT_US |
| 782572002 | SNOMEDCT_US |
| 785624003 | SNOMEDCT_US |
| C0031465 | UMLSCUI |
| 4021030 | VANDF |
| 11518 | MMSL |
| 205556 | MMSL |
| 361870 | MMSL |
| 5490 | MMSL |
| 66426 | MMSL |
| d04070 | MMSL |
| R9K49A127H | UNII |
| 005954 | NDDF |
| 005955 | NDDF |
Pharmaceutical products:
| Product | Category | Ingredients | NDC | Form | Quantity | Route | Marketing | Label |
|---|---|---|---|---|---|---|---|---|
| Sodium Phenylbutyrate | HUMAN PRESCRIPTION DRUG LABEL | 1 | 42794-086 | POWDER | 0.94 g | ORAL | ANDA | 14 sections |
| Sodium Phenylbutyrate | HUMAN PRESCRIPTION DRUG LABEL | 1 | 49884-006 | POWDER | 0.94 g | ORAL | ANDA | 21 sections |
| Sodium Phenylbutyrate | HUMAN PRESCRIPTION DRUG LABEL | 1 | 49884-006 | POWDER | 0.94 g | ORAL | ANDA | 21 sections |
| Sodium Phenylbutyrate | HUMAN PRESCRIPTION DRUG LABEL | 1 | 49884-170 | TABLET | 500 mg | ORAL | ANDA | 12 sections |
| sodium phenylbutyrate | HUMAN PRESCRIPTION DRUG LABEL | 1 | 68462-853 | TABLET | 500 mg | ORAL | ANDA | 20 sections |
| PHEBURANE | HUMAN PRESCRIPTION DRUG LABEL | 1 | 71770-210 | PELLET | 483 mg | ORAL | NDA | 28 sections |
| PHEBURANE | HUMAN PRESCRIPTION DRUG LABEL | 1 | 71770-210 | PELLET | 483 mg | ORAL | NDA | 28 sections |
| RELYVRIO | HUMAN PRESCRIPTION DRUG LABEL | 2 | 73063-035 | POWDER, FOR SUSPENSION | 3 g | ORAL | NDA | 27 sections |
| RELYVRIO | HUMAN PRESCRIPTION DRUG LABEL | 2 | 73063-035 | POWDER, FOR SUSPENSION | 3 g | ORAL | NDA | 27 sections |
| BUPHENYL | HUMAN PRESCRIPTION DRUG LABEL | 1 | 75987-060 | TABLET | 500 mg | ORAL | NDA | 22 sections |
| BUPHENYL | HUMAN PRESCRIPTION DRUG LABEL | 1 | 75987-060 | TABLET | 500 mg | ORAL | NDA | 22 sections |
| BUPHENYL | HUMAN PRESCRIPTION DRUG LABEL | 1 | 75987-060 | TABLET | 500 mg | ORAL | NDA | 22 sections |
| Buphenyl | HUMAN PRESCRIPTION DRUG LABEL | 1 | 75987-070 | POWDER | 0.94 g | ORAL | NDA | 22 sections |
| Buphenyl | HUMAN PRESCRIPTION DRUG LABEL | 1 | 75987-070 | POWDER | 0.94 g | ORAL | NDA | 22 sections |
| Buphenyl | HUMAN PRESCRIPTION DRUG LABEL | 1 | 75987-070 | POWDER | 0.94 g | ORAL | NDA | 22 sections |