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FIGURE: 1 / 3
PCCA Antibody (H00005095-B01P) in WB
Western Blot analysis of PCCA expression in transfected 293T cell line (H00005095-T01) by PCCA MaxPab polyclonal antibody.
Lane 1: PCCA transfected lysate(77.33 KDa).
Lane 2: Non-transfected lysate.
Lane 1: PCCA transfected lysate(77.33 KDa).
Lane 2: Non-transfected lysate.
Product Details
H00005095-B01P
Product Specifications
Species Reactivity
Human
Host/Isotype
Mouse
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
PCCA (AAH00140.1, 1 a.a. approximately 703 a.a) full-length human protein.
Conjugate
Unconjugated
Form
Liquid
Concentration
See Label
Purification
Affinity chromatography
Storage buffer
PBS, pH 7.4
Contains
no preservative
Storage conditions
-20° C, Avoid Freeze/Thaw Cycles
Product Specific Information
Sequence of this protein is as follows: MLSAALRTLK HVLYYSRQCL MVSRNLGSVG YDPNEKTFDK ILVANRGEIA CRVIRTCKKM GIKTVAIHSD VDASSVHVKM ADEAVCVGPA PTSKSYLNMD AIMEAIKKTR AQAVHPGYGF LSENKEFARC LAAEDVVFIG PDTHAIQAMG DKIESKLLAK KAEVNTIPGF DGVVKDAEEA VRIAREIGYP VMIKASAGGG GKGMRIAWDD EETRDGFRLS SQEAASSFGD DRLLIEKFID NPRHIEIQVL GDKHGNALWL NERECSIQRR NQKVVEEAPS IFLDAETRRA MGEQAVALAR AVKYSSAGTV EFLVDSKKNF YFLEMNTRLQ VEHPVTECIT GLDLVQEMIR VAKGYPLRHK QADIRINGWA VECRVYAEDP YKSFGLPSIG RLSQYQEPLH LPGVRVDSGI QPGSDISIYY DPMISKLITY GSDRTEALKR MADALDNYVI RGVTHNIALL REVIINSRFV KGDISTKFLS DVYPDGFKGH MLTKSEKNQL LAIASSLFVA FQLRAQHFQE NSRMPVIKPD IANWELSVKL HDKVHTVVAS NNGSVFSVEV DGSKLNVTST WNLASPLLSV SVDGTQRTVQ CLSREAGGNM SIQFLGTVYK VNILTRLAAE LNKFMLEKVT EDTSSVLRSP MPGVVVAVSV KPGDAVAEGQ EICVIEAMKM QNSMTAGKTG TVKSVHCQAG DTVGEGDLLV ELE
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.
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Bioinformatics
Protein Aliases: pccA complementation group; PCCase alpha subunit; PCCase subunit alpha; propanoyl-CoA:carbon dioxide ligase alpha subunit; Propanoyl-CoA:carbon dioxide ligase subunit alpha; propionyl CoA carboxylase, alpha polypeptide; propionyl Coenzyme A carboxylase, alpha polypeptide; Propionyl-CoA carboxylase alpha chain, mitochondrial; RP11-151A6.1
Gene Aliases: PCCA
UniProt ID: (Human) P05165
Entrez Gene ID: (Human) 5095
Molecular Function:
ligase
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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