Item no. |
ABC-AP7112a |
Manufacturer |
Abcepta
|
Amount |
400 ul |
Category |
|
Type |
Antibody Primary |
Format |
Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS. |
Applications |
WB, IHC-P |
Specific against |
other |
Host |
Rabbit |
Isotype |
Ig |
ECLASS 10.1 |
32160702 |
ECLASS 11.0 |
32160702 |
UNSPSC |
12352203 |
Similar products |
mitochondrial, BCKDK, [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, Branched-chain alpha-ketoacid dehydrogenase kinase, BCKD-kinase, BCKDHKIN |
Available |
|
Primary Accession |
O14874 |
Antigen Type |
Synthetic Peptide |
Application |
IHC-P, WB |
Bio Background |
The second major step in the catabolism of the branched-chain amino acids, isoleucine, leucine, and valine, is irreversibly catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD), an inner-mitochondrial enzyme complex composed of 3 catalytic components: a branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). The complex also contains 2 enzymes that regulated the state of activity of the BCKD complex: a kinase (BCKDK), and a phosphorylase. The ubiquitiously expressed kinase contains 1 histidine kinase domain. Maple syrup urine disease (MSUD) is a pathology secondary to an enzyme defect in the catabolic pathway of leucine, isoleucine, and valine. Accumulation of these amino acids and their corresponding keto acids results in encephalopathy and progressive neurodegeneration in infants not treated for MSUD. |
Bio References |
Chang, C.F., et al., J. Biol. Chem. 277(18):15865-15873 (2002).Popov, K.M., et al., J. Biol. Chem. 267(19):13127-13130 (1992).Zneimer, S.M., et al., Genomics 10(3):740-747 (1991). |
Clonality |
Polyclonal |
Clone/Animal Names |
RB7751 |
Gene ID |
10295 |
Gene Name |
BCKDK |
Subtitle |
Purified Rabbit Polyclonal Antibody (Pab) |
Reactivity |
H |
Legend image 1 |
Western blot analysis of BCKDK (arrow) using rabbit polyclonal BCKDK Antibody (Center) (Cat.#AP7112a). 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected with the BCKDK gene (Lane 2) (Origene Technologies). |
Type image 1 |
WB |
Dilution image 1 |
1:1000 |
Legend image 2 |
Formalin-fixed and paraffin-embedded human cancer tissue reacted with the primary antibody, which was peroxidase-conjugated to the secondary antibody, followed by AEC staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated. BC = breast carcinoma; HC = hepatocarcinoma. |
Type image 2 |
IHC-P |
Dilution image 2 |
1:50~100 |
Other Accession |
Q00972; O55028 |
Predicted |
M, Rat |
Isotype |
Rabbit Ig |
Calculated MW |
46360 |
Antigen Region |
120-151 |
Antigen Source |
HUMAN |
Target/Specificity |
This BCKDK antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 120-151 amino acids from the Central region of human BCKDK. |
NCBI Accession |
NP_001116429.1; NP_005872.2 |
Note: The presented information and documents (Manual, Product Datasheet, Safety Datasheet and Certificate of Analysis) correspond to our latest update and should serve for orientational purpose only. We do not guarantee the topicality. We would kindly ask you to make a request for specific requirements, if necessary.
All products are intended for research use only (RUO). Not for human, veterinary or therapeutic use.