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Invitrogen
PCCA Polyclonal Antibody
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FIGURE: 1 / 1
PCCA Antibody (PA5-97219) in WB
Product Details
PA5-97219
Species Reactivity
Host/Isotype
Class
Type
Immunogen
Conjugate
Form
Concentration
Purification
Storage buffer
Contains
Storage conditions
Shipping conditions
RRID
Product Specific Information
Immunogen sequence: ISKLITYGSD RTEALKRMAD ALDNYVIRGV THNIALLREV IINSRFVKGD ISTKFLSDVY PDGFKGHMLT KSEKNQLLAI ASSLFVAFQL RAQHFQENSR MPVIKPDIAN WELSVKLHDK VHTVVASNNG SVFSVEVDGS KLNVTSTWNL ASPLLSVSVD GTQRTVQCLS REAGGNMSIQ FLGTVYKVNI LTRLAAELNK FMLEKVTEDT SSVLRSPMPG VVVAVSVKPG DAVAEGQEIC VIEAMKMQNS MTAGKTGTVK SVHCQAGDTV GEGDLLVELE; Positive Samples: Mouse pancreas, Mouse liver, Mouse brain, Mouse kidney, Rat kidney; Cellular Location: Mitochondrion matrix
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 propionic acidemia type I (PA-1). 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.
References (0)
Bioinformatics
Protein Aliases: PCCase alpha subunit; PCCase subunit alpha; propanoyl-CoA:carbon dioxide ligase alpha subunit; Propanoyl-CoA:carbon dioxide ligase subunit alpha; propionyl CoA-carboxylase alpha; Propionyl Coenzyme A carboxylase alpha polypeptide; Propionyl-CoA carboxylase alpha chain, mitochondrial; propionyl-coenzyme A carboxylase, alpha polypeptide; RP11-151A6.1
Gene Aliases: C79630; Pcca
UniProt ID: (Rat) P14882, (Mouse) Q91ZA3
Entrez Gene ID: (Rat) 687008, (Mouse) 110821
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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