产品详情
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Product Name
BCKDHB Rabbit pAb
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Gene ID
594
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SwissProt ID
P21953
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Gene Name
BCKDHB
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Alternative Names
E1B; BCKDE1B; BCKDH E1-beta; BCKDHB
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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.
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Research Field
Signal Transduction
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Product Categories
Primary antibody
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Host
Rabbit
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Reactivity
Mouse, Rat
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Application
WB
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Dilution Ratio
WB: 1/500-1/1000
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Molecular Weight
Calculated MW:43kDa
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Clonality
Polyclonal Antibody
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Clonality No.
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Isotype
IgG
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Immunogen
Recombinant fusion protein
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Purification
Affinity Purified
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Conjugation
Unconjugated
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Modification
Unmodified
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Form
Liquid
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Buffer System
Liquid in PBS with 0.02% sodium azide,50% glycerol,pH7.3.
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Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
应用缩写
WB:蛋白质印迹法IHC-P:石蜡切片样本的免疫组织化学法IHC-F:冰冻切片样本的免疫组织化学法ICC/IF:免疫细胞荧光 IP:免疫沉淀法FC:流式细胞术ChIP:染色质免疫沉淀法ELISA:酶联免疫吸附测定
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