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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.
产品信息
H00005095-B01P
产品规格
种属反应
Human
宿主/亚型
Mouse
/ IgG
分类
Polyclonal
类型
Antibody
抗原
PCCA (AAH00140.1, 1 a.a. approximately 703 a.a) full-length human protein.
偶联物
形式
Liquid
浓度
See Label
纯化类型
Affinity chromatography
保存液
PBS, pH 7.4
内含物
no preservative
保存条件
-20° C, Avoid Freeze/Thaw Cycles
运输条件
Ambient (domestic); Wet ice (international)
产品详细信息
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
靶标信息
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.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: 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
基因别名: PCCA
UniProt ID: (Human) P05165
Entrez Gene ID: (Human) 5095
