Anti-BCKDHB Antibody (CAB10533)
- SKU:
- CAB10533
- Product type:
- Antibody
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
| Antibody Name: | Anti-BCKDHB Antibody |
| Antibody SKU: | CAB10533 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB |
| Reactivity: | Mouse |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 183-392 of human BCKDHB (NP_898871.1). |
| Application: | WB |
| Recommended Dilution: | WB 1:500 - 1:2000 |
| Reactivity: | Mouse |
| Positive Samples: | Mouse pancreas |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 183-392 of human BCKDHB (NP_898871.1). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | RSPW GCVG HGAL YHSQ SPEA FFAH CPGI KVVI PRSP FQAK GLLL SCIE DKNP CIFF EPKI LYRA AAEE VPIE PYNI PLSQ AEVI QEGS DVTL VAWG TQVH VIRE VASM AKEK LGVS CEVI DLRT IIPW DVDT ICKS VIKT GRLL ISHE APLT GGFA SEIS STVQ EECF LNLE APIS RVCG YDTP FPHI FEPF YIPD KWKC YDAL RKMI NY |
| Gene ID: | 594 |
| Uniprot: | P21953 |
| Cellular Location: | Mitochondrion matrix |
| Calculated MW: | 23kDa/43kDa |
| Observed MW: | 43kDa |
| Synonyms: | BCKDHB, BCKDE1B, BCKDH E1-beta, E1B, BCKDHE1-beta |
| Background: | This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene 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. Alternative splicing at this locus results in multiple transcript variants. |
| UniProt Protein Function: | BCKDHB: The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Defects in BCKDHB are the cause of maple syrup urine disease type IB (MSUD1B). MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine. |
| UniProt Protein Details: | Protein type:Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 1.2.4.4; Mitochondrial; Oxidoreductase Chromosomal Location of Human Ortholog: 6q14.1 Cellular Component: mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix; mitochondrion Molecular Function:carboxy-lyase activity; protein binding Biological Process: branched chain family amino acid catabolic process; glyoxylate metabolic process Disease: Maple Syrup Urine Disease |
| NCBI Summary: | This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene 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. Alternative splicing at this locus results in multiple transcript variants. [provided by RefSeq, Jan 2016] |
| UniProt Code: | P21953 |
| NCBI GenInfo Identifier: | 129034 |
| NCBI Gene ID: | 594 |
| NCBI Accession: | P21953.2 |
| UniProt Secondary Accession: | P21953,Q5T2J3, Q9BQL0, |
| UniProt Related Accession: | P21953 |
| Molecular Weight: | 43kDa |
| NCBI Full Name: | 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial |
| NCBI Synonym Full Names: | branched chain keto acid dehydrogenase E1 subunit beta |
| NCBI Official Symbol: | BCKDHB |
| NCBI Official Synonym Symbols: | E1B; BCKDE1B; BCKDH E1-beta |
| NCBI Protein Information: | 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial |
| UniProt Protein Name: | 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial |
| UniProt Synonym Protein Names: | Branched-chain alpha-keto acid dehydrogenase E1 component beta chain; BCKDE1B; BCKDH E1-beta |
| UniProt Gene Name: | BCKDHB |
 | Western blot analysis of extracts of mouse pancreas, using BCKDHB antibody (CAB10533) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 1s. |
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