PAH Antibody, Biotin conjugated (PACO28797)
- SKU:
- PACO28797
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- Biotin
Frequently bought together:
Description
抗体名: | PAH Antibody, Biotin conjugated (PACO28797) |
抗体コード: | PACO28797 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human Phenylalanine-4-hydroxylase protein (2-452AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Biotin |
バックグラウンド: | cytosol, extracellular exosome, phenylalanine 4-monooxygenase activity, catecholamine biosynthetic process, cellular amino acid, biosynthetic process, cellular nitrogen compound metabolic process, L-phenylalanine catabolic process, neurotransmitter biosynthetic process, small molecule metabolic process |
シノニム: | Phenylalanine-4-hydroxylase (PAH) (EC 1.14.16.1) (Phe-4-monooxygenase), PAH |
UniProt Protein Function: | PAH: phenylalanine hydroxylase that is the rate-limiting step in phenylalanine catabolism. Belongs to the biopterin-dependent aromatic amino acid hydroxylase family. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria. |
UniProt Protein Details: | Protein type:EC 1.14.16.1; Amino Acid Metabolism - phenylalanine, tyrosine and tryptophan biosynthesis; Oxidoreductase Chromosomal Location of Human Ortholog: 12q22-q24.2 Cellular Component: cytosol Molecular Function:amino acid binding; iron ion binding; phenylalanine 4-monooxygenase activity Biological Process: L-phenylalanine catabolic process; catecholamine biosynthetic process; amino acid biosynthetic process; neurotransmitter biosynthetic process Disease: Phenylketonuria |
NCBI Summary: | PAH encodes the enzyme phenylalanine hydroxylase that is the rate-limiting step in phenylalanine catabolism. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria. [provided by RefSeq, Jul 2008] |
UniProt Code: | P00439 |
NCBI GenInfo Identifier: | 129973 |
NCBI Gene ID: | 5053 |
NCBI Accession: | P00439.1 |
UniProt Secondary Accession: | P00439,Q16717, Q8TC14, |
UniProt Related Accession: | P00439 |
Molecular Weight: | 452 |
NCBI Full Name: | Phenylalanine-4-hydroxylase |
NCBI Synonym Full Names: | phenylalanine hydroxylase |
NCBI Official Symbol: | PAH |
NCBI Official Synonym Symbols: | PH; PKU; PKU1 |
NCBI Protein Information: | phenylalanine-4-hydroxylase; phe-4-monooxygenase; phenylalanine 4-monooxygenase |
UniProt Protein Name: | Phenylalanine-4-hydroxylase |
UniProt Synonym Protein Names: | Phe-4-monooxygenase |
UniProt Gene Name: | PAH |
UniProt Entry Name: | PH4H_HUMAN |