| 产品详情 |
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| Antigenic Specificity | PCCA |
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| Clone | polyclonal |
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| Host Species | Rabbit |
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| Reactive Species | human |
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| Isotype | n/a |
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| Format | purified |
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| Size | 100 µl |
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| Concentration | n/a |
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| Applications | Immunohistochemistry: 1:50-1:200, Western Blot: 0.4 µg/mL |
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| Reviews / Ratings | If you have used this antibody, please help fellow researchers by submitting reviews to pAbmAbs and antYbuddY. |
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| Description | PCCA Polyclonal Antibody. 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. |
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| Immunogen | n/a |
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| Other Names | PCCase alpha subunit, pccA complementation group, propanoyl-CoA:carbon dioxide ligase alpha subunit, propionyl Coenzyme A carboxylase, alpha polypeptide, propionyl-CoA carboxylase alpha chain, mitochondrial |
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| Gene, Accession # | Entrez: 5095; Uniprot: P05165 |
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| Catalog # | PA5-61590 |
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| Price | |
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| Order / More Info | PCCA Antibody from INVITROGEN ANTIBODIES |
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| Product Specific References | n/a |
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