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Gene ID: 593 | BCKDHA Antibody Resource


BCKDHA Antibody Resource
Gene Full Name: 
branched chain keto acid dehydrogenase E1, alpha polypeptide
Uniprot: 
P12694
Protein Name: 
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Nucleotide: 
NM_000709
Alias: 
MSU;MSUD1;OVD1A;BCKDE1A;
Ensembl: 
ENSG00000248098
Location: 
19q13.1-q13.2
HPRD: 
02009
NCBI Gene: 
593
MIM: 
608348

Summary: 
The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Sep 2009]
Function: 
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Subcellular location: 
Mitochondrion matrix.
PTM: 
Acetylation;
Disease: 
Maple syrup urine disease 1A (MSUD1A) [MIM:248600]: A metabolic disorder due to an enzyme defect in the catabolic pathway of the branched-chain amino acids leucine, isoleucine, and valine. Accumulation of these 3 amino acids and their corresponding keto acids leads to encephalopathy and progressive neurodegeneration. Clinical features include mental and physical retardation, feeding problems, and a maple syrup odor to the urine. The keto acids of the branched-chain amino acids are present in the urine. If untreated, maple syrup urine disease can lead to seizures, coma, and death. The disease is often classified by its pattern of signs and symptoms. The most common and severe form of the disease is the classic type, which becomes apparent soon after birth. Variant forms of the disorder become apparent later in infancy or childhood and are typically milder, but they still involve developmental delay and other medical problems if not treated. Note: The disease is caused by mutations affecting the gene represented in this entry.
Biotechnological use: 

Antibodies
TypeCatalogNameClonalitySpecies ReactivityApplicationSizePriceMore Info
Polyclonal Antibody ARP56119_P050 BCKDHA antibody - N-terminal region (ARP56119_P050) Polyclonal Bovine, Horse, Human, Mouse, Pig, Rabbit, Rat, Sheep, Zebrafish WB 50ug $289 Details
Polyclonal Antibody OAAB03929 BCKDHA antibody - C-terminal region (OAAB03929) Human WB, IHC, ELISA 400ul $289 Details
Others OOPA00107 BCKDHA Human - Recombinant Human Branched Chain keto Acid Dehydrogenase E1, Alpha (1ug) 1ug $100 Details
Polyclonal Antibody ARP56120_P050 BCKDHA Antibody (ARP56120_P050) Polyclonal Bovine, Dog, Guinea pig, Horse, Human, Mouse, Pig, Rabbit, Rat, Sheep, Zebrafish Not tested 50 ug $125 Details

Show all BCKDHA antibodies


Product Images
BCKDHA antibody - N-terminal region (ARP56119_P050) in Hum. Adult Placenta cells using Western Blot

Sample Type: Hum. Adult Placenta

BCKDHA antibody - N-terminal region (ARP56119_P050) in Human MCF-7 cells using Western Blot

Sample Type: Human MCF-7

BCKDHA antibody - C-terminal region (OAAB03929) in human lung carcinoma cells using Immunohistochemistry

Sample Type: human lung carcinoma

BCKDHA antibody - C-terminal region (OAAB03929) in Jurkat cells using Western Blot

Sample Type: Jurkat


BCKDHA Protocols

Publications

Related Gene Trend Categories

Species
CatalogPredicted Species & TargetTarget ReferencePredicted Homology
ARP56120 Marinobacter hydrocarbonoclasticus ATCC 49840 BCKDHA antibody H8W5R3 86.0%
ARP56119 Bovine BCKDHA antibody; Bos taurus BCKDHA antibody F1N5F2 100.0%
ARP56120 Bovine BCKDHA antibody; Bos taurus BCKDHA antibody F1N5F2 100.0%
ARP56119 Bovine BCKDHA antibody; Bos taurus BCKDHA antibody P11178 100.0%
ARP56120 Bovine BCKDHA antibody; Bos taurus BCKDHA antibody P11178 100.0%
ARP56119 Bovine BCKDHA antibody; Bos taurus BCKDHA antibody Q2TBT9 100.0%
ARP56120 Bovine BCKDHA antibody; Bos taurus BCKDHA antibody Q2TBT9 100.0%
ARP56119 Cat BCKDHA antibody; Felis catus BCKDHA antibody M3W423 100.0%
ARP56120 Cat BCKDHA antibody; Felis catus BCKDHA antibody M3W423 100.0%
ARP56119 Chimpanzee BCKDHA antibody; Pan troglodytes BCKDHA antibody A5A6H9 100.0%
ARP56120 Chimpanzee BCKDHA antibody; Pan troglodytes BCKDHA antibody A5A6H9 100.0%
ARP56119 Chimpanzee BCKDHA antibody; Pan troglodytes BCKDHA antibody H2QGE1 100.0%
ARP56120 Chimpanzee BCKDHA antibody; Pan troglodytes BCKDHA antibody H2QGE1 100.0%
ARP56119 Crab-eating macaque BCKDHA antibody; Macaca fascicularis BCKDHA antibody Q8HXY4 100.0%
ARP56120 Crab-eating macaque BCKDHA antibody; Macaca fascicularis BCKDHA antibody Q8HXY4 100.0%
ARP56119 Dog BCKDHA antibody; Canis familiaris BCKDHA antibody E2RPW4 100.0%
ARP56120 Dog BCKDHA antibody; Canis familiaris BCKDHA antibody E2RPW4 100.0%
ARP56119 Dog BCKDHA antibody; Canis familiaris BCKDHA antibody F1PI86 100.0%
ARP56120 Dog BCKDHA antibody; Canis familiaris BCKDHA antibody F1PI86 100.0%
ARP44955 Dog BCKDHA antibody; Canis familiaris BCKDHA antibody E2RPW4 92.0%
ARP44955 Dog BCKDHA antibody; Canis familiaris BCKDHA antibody F1PI86 92.0%
ARP56119 European domestic ferret BCKDHA antibody; Mustela putorius furo BCKDHA antibody M3Z1S4 100.0%
ARP56120 European domestic ferret BCKDHA antibody; Mustela putorius furo BCKDHA antibody M3Z1S4 100.0%
ARP56119 Horse BCKDHA antibody; Equus caballus BCKDHA antibody F6REC8 100.0%
ARP56120 Horse BCKDHA antibody; Equus caballus BCKDHA antibody F6REC8 100.0%
ARP56119 Horse BCKDHA antibody; Equus caballus BCKDHA antibody F6SJ93 100.0%
ARP56120 Horse BCKDHA antibody; Equus caballus BCKDHA antibody F6SJ93 100.0%
ARP44957 Horse BCKDHA antibody; Equus caballus BCKDHA antibody F6SJ93 79.0%
ARP56119 Human BCKDHA antibody; Homo sapiens BCKDHA antibody B4DP47 100.0%
ARP56120 Human BCKDHA antibody; Homo sapiens BCKDHA antibody B4DP47 100.0%
ARP56119 Human BCKDHA antibody; Homo sapiens BCKDHA antibody F5GXU9 100.0%
ARP56120 Human BCKDHA antibody; Homo sapiens BCKDHA antibody F5GXU9 100.0%
ARP56119 Human BCKDHA antibody; Homo sapiens BCKDHA antibody H0YH31 100.0%
ARP56119 Human BCKDHA antibody; Homo sapiens BCKDHA antibody P12694 100.0%
ARP56120 Human BCKDHA antibody; Homo sapiens BCKDHA antibody P12694 100.0%
ARP69567 Human BCKDHA antibody; Homo sapiens BCKDHA antibody H0YH31 86.0%
ARP56119 Mouse BCKDHA antibody; Mus musculus BCKDHA antibody Q99L69 100.0%
ARP56120 Mouse BCKDHA antibody; Mus musculus BCKDHA antibody Q99L69 100.0%
ARP56119 Mouse BCKDHA antibody; Mus musculus BCKDHA antibody P50136 100.0%
ARP56120 Mouse BCKDHA antibody; Mus musculus BCKDHA antibody P50136 100.0%
ARP56119 Mouse BCKDHA antibody; Mus musculus BCKDHA antibody Q3U3J1 100.0%
ARP56120 Mouse BCKDHA antibody; Mus musculus BCKDHA antibody Q3U3J1 100.0%
ARP56119 Rabbit BCKDHA antibody; Oryctolagus cuniculus BCKDHA antibody G1U5X6 100.0%
ARP56120 Rabbit BCKDHA antibody; Oryctolagus cuniculus BCKDHA antibody G1U5X6 100.0%
ARP56119 Rat BCKDHA antibody; Rattus norvegicus BCKDHA antibody B1WBN3 100.0%
ARP56120 Rat BCKDHA antibody; Rattus norvegicus BCKDHA antibody B1WBN3 100.0%
ARP56119 Rat BCKDHA antibody; Rattus norvegicus BCKDHA antibody P11960 100.0%
ARP56120 Rat BCKDHA antibody; Rattus norvegicus BCKDHA antibody P11960 100.0%
ARP56120 Rat BCKDHA antibody; Rattus norvegicus BCKDHA antibody Q5EB89 100.0%
ARP56120 Spotted green pufferfish BCKDHA antibody; Tetraodon nigroviridis BCKDHA antibody Q4RRE6 93.0%
ARP56119 Spotted green pufferfish BCKDHA antibody; Tetraodon nigroviridis BCKDHA antibody Q4RRE6 79.0%
ARP56120 Western clawed frog BCKDHA antibody; Xenopus tropicalis BCKDHA antibody A0JPC4 100.0%
ARP56119 Western clawed frog BCKDHA antibody; Xenopus tropicalis BCKDHA antibody F7BVG6 100.0%
ARP56120 Western clawed frog BCKDHA antibody; Xenopus tropicalis BCKDHA antibody F7BVG6 100.0%
ARP56119 White-tufted-ear marmoset BCKDHA antibody; Callithrix jacchus BCKDHA antibody F6XSY5 100.0%
ARP56120 White-tufted-ear marmoset BCKDHA antibody; Callithrix jacchus BCKDHA antibody F6XSY5 100.0%
ARP56119 White-tufted-ear marmoset BCKDHA antibody; Callithrix jacchus BCKDHA antibody F6XU89 100.0%
ARP56120 White-tufted-ear marmoset BCKDHA antibody; Callithrix jacchus BCKDHA antibody F6XU89 100.0%
ARP56119 White-tufted-ear marmoset BCKDHA antibody; Callithrix jacchus BCKDHA antibody F6XUA6 100.0%
ARP56120 White-tufted-ear marmoset BCKDHA antibody; Callithrix jacchus BCKDHA antibody F6XUA6 100.0%
ARP56119 White-tufted-ear marmoset BCKDHA antibody; Callithrix jacchus BCKDHA antibody F6YFU7 100.0%
ARP56120 White-tufted-ear marmoset BCKDHA antibody; Callithrix jacchus BCKDHA antibody F6YFU7 100.0%
ARP56119 Zebrafish BCKDHA antibody; Danio rerio BCKDHA antibody Q4VBU0 93.0%
ARP56120 Zebrafish BCKDHA antibody; Danio rerio BCKDHA antibody Q4VBU0 93.0%

Tissue & Cell Line Gene Expression Data
Data SourceProbesetSampleAvgMeanMore Info
BioGPS Gene Atlas202331_at721_B_lymphoblasts118.85030.280Details
BioGPS Gene Atlas202331_atAdipocyte16.40030.280Details
BioGPS Gene Atlas202331_atAdrenalCortex21.30030.280Details
BioGPS Gene Atlas202331_atAdrenalgland47.50030.280Details
BioGPS Gene Atlas202331_atAmygdala15.80030.280Details
BioGPS Gene Atlas202331_atAppendix16.55030.280Details
BioGPS Gene Atlas202331_atAtrioventricularNode9.85030.280Details
BioGPS Gene Atlas202331_atBDCA4+_DentriticCells103.25030.280Details
BioGPS Gene Atlas202331_atBonemarrow30.55030.280Details
BioGPS Gene Atlas202331_atBronchialEpithelialCells16.65030.280Details
BioGPS Gene Atlas202331_atCD105+_Endothelial15.00030.280Details
BioGPS Gene Atlas202331_atCD14+_Monocytes61.45030.280Details
BioGPS Gene Atlas202331_atCD19+_BCells(neg._sel.)24.85030.280Details
BioGPS Gene Atlas202331_atCD33+_Myeloid48.65030.280Details
BioGPS Gene Atlas202331_atCD34+19.80030.280Details
BioGPS Gene Atlas202331_atCD4+_Tcells32.65030.280Details
BioGPS Gene Atlas202331_atCD56+_NKCells84.50030.280Details
BioGPS Gene Atlas202331_atCD71+_EarlyErythroid11.85030.280Details
BioGPS Gene Atlas202331_atCD8+_Tcells23.60030.280Details
BioGPS Gene Atlas202331_atCardiacMyocytes17.20030.280Details
BioGPS Gene Atlas202331_atCaudatenucleus14.60030.280Details
BioGPS Gene Atlas202331_atCerebellum24.15030.280Details
BioGPS Gene Atlas202331_atCerebellumPeduncles20.80030.280Details
BioGPS Gene Atlas202331_atCiliaryGanglion9.45030.280Details
BioGPS Gene Atlas202331_atCingulateCortex17.15030.280Details
BioGPS Gene Atlas202331_atColorectaladenocarcinoma23.75030.280Details
BioGPS Gene Atlas202331_atDorsalRootGanglion10.05030.280Details
BioGPS Gene Atlas202331_atFetalThyroid33.30030.280Details
BioGPS Gene Atlas202331_atFetalbrain14.40030.280Details
BioGPS Gene Atlas202331_atFetalliver9.10030.280Details
BioGPS Gene Atlas202331_atFetallung15.65030.280Details
BioGPS Gene Atlas202331_atGlobusPallidus12.60030.280Details
BioGPS Gene Atlas202331_atHeart175.65030.280Details
BioGPS Gene Atlas202331_atHypothalamus19.10030.280Details
BioGPS Gene Atlas202331_atKidney32.50030.280Details
BioGPS Gene Atlas202331_atLeukemia_chronicMyelogenousK-56221.55030.280Details
BioGPS Gene Atlas202331_atLeukemia_promyelocytic-HL-6017.25030.280Details
BioGPS Gene Atlas202331_atLeukemialymphoblastic(MOLT-4)19.65030.280Details
BioGPS Gene Atlas202331_atLiver190.60030.280Details
BioGPS Gene Atlas202331_atLung80.65030.280Details
BioGPS Gene Atlas202331_atLymphnode14.10030.280Details
BioGPS Gene Atlas202331_atLymphoma_burkitts(Daudi)21.80030.280Details
BioGPS Gene Atlas202331_atLymphoma_burkitts(Raji)65.45030.280Details
BioGPS Gene Atlas202331_atMedullaOblongata15.00030.280Details
BioGPS Gene Atlas202331_atOccipitalLobe14.15030.280Details
BioGPS Gene Atlas202331_atOlfactoryBulb13.65030.280Details
BioGPS Gene Atlas202331_atOvary11.70030.280Details
BioGPS Gene Atlas202331_atPancreas22.60030.280Details
BioGPS Gene Atlas202331_atPancreaticIslet26.40030.280Details
BioGPS Gene Atlas202331_atParietalLobe15.10030.280Details
BioGPS Gene Atlas202331_atPituitary19.75030.280Details
BioGPS Gene Atlas202331_atPlacenta17.65030.280Details
BioGPS Gene Atlas202331_atPons13.60030.280Details
BioGPS Gene Atlas202331_atPrefrontalCortex18.00030.280Details
BioGPS Gene Atlas202331_atProstate69.95030.280Details
BioGPS Gene Atlas202331_atSalivarygland10.95030.280Details
BioGPS Gene Atlas202331_atSkeletalMuscle20.95030.280Details
BioGPS Gene Atlas202331_atSkin11.05030.280Details
BioGPS Gene Atlas202331_atSmoothMuscle12.85030.280Details
BioGPS Gene Atlas202331_atSpinalcord20.95030.280Details
BioGPS Gene Atlas202331_atSubthalamicNucleus12.80030.280Details
BioGPS Gene Atlas202331_atSuperiorCervicalGanglion16.10030.280Details
BioGPS Gene Atlas202331_atTemporalLobe16.75030.280Details
BioGPS Gene Atlas202331_atTestis47.10030.280Details
BioGPS Gene Atlas202331_atTestisGermCell15.45030.280Details
BioGPS Gene Atlas202331_atTestisIntersitial12.40030.280Details
BioGPS Gene Atlas202331_atTestisLeydigCell14.55030.280Details
BioGPS Gene Atlas202331_atTestisSeminiferousTubule15.20030.280Details
BioGPS Gene Atlas202331_atThalamus22.25030.280Details
BioGPS Gene Atlas202331_atThymus33.20030.280Details
BioGPS Gene Atlas202331_atThyroid54.80030.280Details
BioGPS Gene Atlas202331_atTongue17.55030.280Details
BioGPS Gene Atlas202331_atTonsil14.95030.280Details
BioGPS Gene Atlas202331_atTrachea16.25030.280Details
BioGPS Gene Atlas202331_atTrigeminalGanglion15.10030.280Details
BioGPS Gene Atlas202331_atUterus9.85030.280Details
BioGPS Gene Atlas202331_atUterusCorpus16.25030.280Details
BioGPS Gene Atlas202331_atWholeBlood32.05030.280Details
BioGPS Gene Atlas202331_atWholebrain33.90030.280Details
BioGPS Gene Atlas202331_atcolon15.15030.280Details
BioGPS Gene Atlas202331_atpineal_day52.96030.280Details
BioGPS Gene Atlas202331_atpineal_night42.72030.280Details
BioGPS Gene Atlas202331_atretina22.42530.280Details
BioGPS Gene Atlas202331_atsmall_intestine15.10030.280Details
BioGPS NCI60202331_at7867.27515.250Details
BioGPS NCI60202331_atA17250.69715.250Details
BioGPS NCI60202331_atA2046.74815.250Details
BioGPS NCI60202331_atA3619.98915.250Details
BioGPS NCI60202331_atA4984.75315.250Details
BioGPS NCI60202331_atA54926.99315.250Details
BioGPS NCI60202331_atACC39.58815.250Details
BioGPS NCI60202331_atACHN7.25915.250Details
BioGPS NCI60202331_atALVA316.84915.250Details
BioGPS NCI60202331_atCAKI17.69015.250Details
BioGPS NCI60202331_atCCRT CEM7.96915.250Details
BioGPS NCI60202331_atCOLO20520.65915.250Details
BioGPS NCI60202331_atDLD19.26515.250Details
BioGPS NCI60202331_atDU14519.92215.250Details
BioGPS NCI60202331_atEKVX41.34515.250Details
BioGPS NCI60202331_atGM1338.88715.250Details
BioGPS NCI60202331_atGM16007.75515.250Details
BioGPS NCI60202331_atGM23134.93415.250Details
BioGPS NCI60202331_atGM23457.55715.250Details
BioGPS NCI60202331_atGM24933.00015.250Details
BioGPS NCI60202331_atGM979.05115.250Details
BioGPS NCI60202331_atHCC299825.73115.250Details
BioGPS NCI60202331_atHCT11612.91215.250Details
BioGPS NCI60202331_atHCT1593.17515.250Details
BioGPS NCI60202331_atHEK 293 T-rex9.44115.250Details
BioGPS NCI60202331_atHEK 293T9.90915.250Details
BioGPS NCI60202331_atHEK29312.34515.250Details
BioGPS NCI60202331_atHELA7.29215.250Details
BioGPS NCI60202331_atHEPG27.50615.250Details
BioGPS NCI60202331_atHL6023.65515.250Details
BioGPS NCI60202331_atHOP626.83815.250Details
BioGPS NCI60202331_atHOP929.74915.250Details
BioGPS NCI60202331_atHS578T6.66215.250Details
BioGPS NCI60202331_atHSG80.61515.250Details
BioGPS NCI60202331_atHT10807.50715.250Details
BioGPS NCI60202331_atHT2927.84515.250Details
BioGPS NCI60202331_atIGROV111.89815.250Details
BioGPS NCI60202331_atIOSE807.52615.250Details
BioGPS NCI60202331_atJURKAT9.94415.250Details
BioGPS NCI60202331_atK5627.79315.250Details
BioGPS NCI60202331_atKM1213.40315.250Details
BioGPS NCI60202331_atLN1843.17015.250Details
BioGPS NCI60202331_atLNCAP6.94515.250Details
BioGPS NCI60202331_atLOX IMVI15.39715.250Details
BioGPS NCI60202331_atM1411.87715.250Details
BioGPS NCI60202331_atMALME 3M7.79415.250Details
BioGPS NCI60202331_atMCF716.10915.250Details
BioGPS NCI60202331_atMD MB2317.04815.250Details
BioGPS NCI60202331_atMDA MB4358.25315.250Details
BioGPS NCI60202331_atMOLT417.21415.250Details
BioGPS NCI60202331_atNCI 4607.68815.250Details
BioGPS NCI60202331_atNCI H2265.53515.250Details
BioGPS NCI60202331_atNCI H2312.46315.250Details
BioGPS NCI60202331_atNCI H322M7.04215.250Details
BioGPS NCI60202331_atNCVADR RES8.78515.250Details
BioGPS NCI60202331_atOVCAR37.72015.250Details
BioGPS NCI60202331_atOVCAR411.34515.250Details
BioGPS NCI60202331_atOVCAR57.45415.250Details
BioGPS NCI60202331_atOVCAR86.01215.250Details
BioGPS NCI60202331_atPANC112.15615.250Details
BioGPS NCI60202331_atRL719.25615.250Details
BioGPS NCI60202331_atRPMI 822624.24815.250Details
BioGPS NCI60202331_atRS1184618.96815.250Details
BioGPS NCI60202331_atRXF3938.78715.250Details
BioGPS NCI60202331_atSAOS210.29015.250Details
BioGPS NCI60202331_atSF2688.79515.250Details
BioGPS NCI60202331_atSF29513.58215.250Details
BioGPS NCI60202331_atSF53912.07115.250Details
BioGPS NCI60202331_atSHSYSY+RA23.76915.250Details
BioGPS NCI60202331_atSHSYSY-RA8.74715.250Details
BioGPS NCI60202331_atSKMEL27.50115.250Details
BioGPS NCI60202331_atSKMEL2811.69915.250Details
BioGPS NCI60202331_atSKMEL510.70015.250Details
BioGPS NCI60202331_atSKOV37.46915.250Details
BioGPS NCI60202331_atSN12C10.11615.250Details
BioGPS NCI60202331_atSNB1927.96115.250Details
BioGPS NCI60202331_atSNB759.84915.250Details
BioGPS NCI60202331_atSR7.34915.250Details
BioGPS NCI60202331_atSW62011.18415.250Details
BioGPS NCI60202331_atT246.43215.250Details
BioGPS NCI60202331_atT3M410.37815.250Details
BioGPS NCI60202331_atTK106.69815.250Details
BioGPS NCI60202331_atU11837.00115.250Details
BioGPS NCI60202331_atU13810.59415.250Details
BioGPS NCI60202331_atU20S14.69215.250Details
BioGPS NCI60202331_atU2517.18615.250Details
BioGPS NCI60202331_atU8747.29815.250Details
BioGPS NCI60202331_atUACC25712.00815.250Details
BioGPS NCI60202331_atUACC6213.78715.250Details
BioGPS NCI60202331_atUO315.23915.250Details
BioGPS NCI60202331_atZR75_19.81815.250Details
BioGPS NCI60202331_atastrocytes7.61715.250Details
BioGPS NCI60202331_athuh-79.20415.250Details