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BCKDK Antibody (Center)

BCKDK Antibody (Center)

Item no.	ABC-AP7112a
Manufacturer	Abcepta
Amount	400 ul
Category	Antibodies Primary Antibodies
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.

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