Thermo Fisher Scientific PCCA Polyclonal Antibody, MaxPab

Applications

• Western Blot (WB)
  Tested Dilution: 1:500–1:1,000

Product Specifications

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 likely a dodecamer composed of six biotin-containing alpha subunits and six beta subunits, located in the mitochondrial matrix.
Defects in PCCA cause propionic acidemia type I (PA-1), a life-threatening disease characterized by episodic vomiting, lethargy, ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.