Cell Biology Antibodies 12

Anti-BCKDK Antibody (CAB8184)

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SKU:
CAB8184
Product Type:
Antibody
Applications:
WB
Applications:
IHC
Applications:
IF
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology

Description

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Antibody Name:BCKDK Rabbit Polyclonal Antibody
Antibody SKU:CAB8184
Antibody Size:20uL, 50uL, 100uL
Application:WB IHC IF
Reactivity:Human, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 153-412 of human BCKDK (NP_005872.2).
Application:WB IHC IF
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:100 IF 1:50 - 1:100
Reactivity:Human, Rat
Positive Samples:22Rv1
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 153-412 of human BCKDK (NP_005872.2).
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:QLVR QLLD DHKD VVTL LAEG LRES RKHI EDEK LVRY FLDK TLTS RLGI RMLA THHL ALHE DKPD FVGI ICTR LSPK KIIE KWVD FARR LCEH KYGN APRV RING HVAA RFPF IPMP LDYI LPEL LKNA MRAT MESH LDTP YNVP DVVI TIAN NDVD LIIR ISDR GGGI AHKD LDRV MDYH FTTA EAST QDPR ISPL FGHL DMHS GAQS GPMH GFGF GLPT SRAY AEYL GGSL QLQS LQGI GTDV YLRL RHID GREE SFRI
Gene ID:10295
Uniprot:O14874
Cellular Location:Mitochondrion, Mitochondrion matrix
Calculated MW:37kDa/41kDa/46kDa
Observed MW:46kDa
Synonyms:BCKDK, BCKDKD, BDK
Background:The branched-chain alpha-ketoacid dehydrogenase complex (BCKD) is an important regulator of the valine, leucine, and isoleucine catabolic pathways. The protein encoded by this gene is found in the mitochondrion, where it phosphorylates and inactivates BCKD. Several transcript variants encoding different isoforms have been found for this gene.
UniProt Protein Function:BCKDK: an atypical protein kinase associated with the mitochondrial matrix. Contains a HATPase_c domain, found in several ATP-binding proteins including protein histidine kinases (PHKs), PHDKs, DNA gyrase B, topoisomerases, heat shock proteins, and DNA mismatch repair proteins. Unlike PHKs, BCK dimerizes through direct interaction of two opposing nucleotide-binding domains. Phosphorylates and inactivates the E1-alpha subunit of the branched-chain ketoacid dehydrogenase (BCKDK), the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Inactivating mutations have been discovered in BCKDK in families with autism, epilepsy, and intellectual disability, a syndrome that may respond to dietary supplementation.
UniProt Protein Details:

Protein type:Mitochondrial; Kinase, protein; Protein kinase, atypical; EC 2.7.11.4; ATYPICAL group; PDHK family

Chromosomal Location of Human Ortholog: 16p11.2

Cellular Component: mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix; mitochondrion

Molecular Function:[3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity; ATP binding; kinase activity; protein binding; protein serine/threonine kinase activity

Biological Process: amino acid catabolic process; branched chain family amino acid catabolic process; phosphorylation; protein amino acid phosphorylation

Disease: Branched-chain Ketoacid Dehydrogenase Kinase Deficiency

NCBI Summary:The branched-chain alpha-ketoacid dehydrogenase complex (BCKD) is an important regulator of the valine, leucine, and isoleucine catabolic pathways. The protein encoded by this gene is found in the mitochondrion, where it phosphorylates and inactivates BCKD. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Dec 2012]
UniProt Code:O14874
NCBI GenInfo Identifier:21431746
NCBI Gene ID:10295
NCBI Accession:O14874.2
UniProt Secondary Accession:O14874,Q6FGL4, Q96G95, Q96IN5, A8MY43,
UniProt Related Accession:O14874
Molecular Weight:41,090 Da
NCBI Full Name:[3-methyl-2-oxobutanoate dehydrogenase
NCBI Synonym Full Names:branched chain ketoacid dehydrogenase kinase
NCBI Official Symbol:BCKDK  
NCBI Official Synonym Symbols:BDK; BCKDKD  
NCBI Protein Information:[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
UniProt Protein Name:[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
UniProt Synonym Protein Names:Branched-chain alpha-ketoacid dehydrogenase kinase; BCKD-kinase; BCKDHKIN
UniProt Gene Name:BCKDK  
UniProt Entry Name:BCKD_HUMAN
Anti-BCKDK Polyclonal Antibody (CAB8184)Western blot analysis of extracts of 22Rv1 cells, using BCKDK antibody (CAB8184) 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: 90s.
Anti-BCKDK Polyclonal Antibody (CAB8184)Immunohistochemistry of paraffin-embedded rat lung using BCKDK antibody (CAB8184) at dilution of 1:100 (40x lens).
Anti-BCKDK Polyclonal Antibody (CAB8184)Immunohistochemistry of paraffin-embedded rat kidney using BCKDK antibody (CAB8184) at dilution of 1:100 (40x lens).
Anti-BCKDK Polyclonal Antibody (CAB8184)Immunohistochemistry of paraffin-embedded human liver damage using BCKDK antibody (CAB8184) at dilution of 1:100 (40x lens).
Anti-BCKDK Polyclonal Antibody (CAB8184)Immunofluorescence analysis of U2OS cells using BCKDK antibody (CAB8184) at dilution of 1:100. Blue: DAPI for nuclear staining.
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