2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB) Antibody
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Catalogue No: abx025837
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US$326.25
(Size: 80 µl)
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Datasheet SDS
Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems.
Research Articles on 2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB)
| Target | 2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB) |
| Clonality | Polyclonal |
| Reactivity | Human |
| Tested Applications | ELISA, WB, IHC, IF/ICC, FCM |
| Host | Rabbit |
| Recommended dilutions | WB: 1/1000, IHC-P: 1/500, IF/ICC: 1/25, FCM: 1/10 - 1/50. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user. |
| Conjugation | Unconjugated |
| Immunogen | KLH-conjugated synthetic peptide between 41-70 amino acids from the N-terminal region of human BCKDHB. |
| Isotype | IgG |
| Form | Liquid |
| Purification | Purified through a protein A column, followed by peptide affinity purification. |
| Storage | Aliquot and store at 2-8°C for up to 2 weeks, or at -20°C for long term storage. Avoid repeated freeze/thaw cycles. |
| Shelf Life | 12 months. |
| UniProt Primary AC | P21953 (UniProt, ExPASy) |
| UniProt Secondary AC | Q5T2J3, Q9BQL0 |
| UniProt Entry Name | ODBB_HUMAN |
| Gene Symbol | BCKDHB |
| KEGG | hsa:594 |
| String | 9606.ENSP00000318351 |
| Molecular Weight | Calculated MW: 43.1 kDa |
| Buffer | PBS containing 0.09% sodium azide. |
| Availability | Shipped within 5-10 working days. |
| Note | THIS PRODUCT IS FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC, THERAPEUTIC OR COSMETIC PROCEDURES. NOT FOR HUMAN OR ANIMAL CONSUMPTION. |