Cell Biology Antibodies 2

Anti-BCKDHB Antibody (CAB10533)

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SKU:
CAB10533
Product Type:
Antibody
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology

Description

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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  
Anti-BCKDHB Antibody (CAB10533)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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