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Product Details
H00131566-M01
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human
Host/Isotype
Mouse
/ IgG2a, kappa
Class
Monoclonal
Type
Antibody
Clone
3G10
Immunogen
DCBLD2 (NP_563615.3, 80 a.a. approximately 164 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Conjugate
Form
Liquid
Concentration
0.5-1 mg/mL
Purification
Protein A
Storage buffer
PBS, pH 7.4
Contains
no preservative
Storage conditions
-20° C, Avoid Freeze/Thaw Cycles
Shipping conditions
Wet ice
Product Specific Information
Immunogen sequence: ESGTLTSINY PQTYPNSTVC EWEIRVKMGE RVRIKFGDFD IEDSDSCHFN YLRIYNGIGV SRTEIGKYCG LGLQMNHSIE SKGNE
Target Information
This protein is a key enzyme in the catabolic pathway of odd-chain fatty acids, isoleucine, threonine, methionine, and valine. It is probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits. The protein is located in the mitochondrial matrix. Defects in PCCA are the cause o fpropionic acidemia type I (PA-1) [MIM:606054]. PA-1 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, develop-mental retardation, and intolerance to protein.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: 1700055P21Rik; coagulation factor V/VIII-homology domains protein 1; CUB, LCCL and coagulation factor V/VIII-homology domains protein 1; discoidin; Discoidin, CUB and LCCL domain-containing protein 2; Endothelial and smooth muscle cell-derived neuropilin-like protein
Gene Aliases: CLCP1; DCBLD2; ESDN
UniProt ID: (Human) Q96PD2
Entrez Gene ID: (Human) 131566
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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