Metabolism Antibodies 1
Anti-PAH Antibody (CAB1559)
- SKU:
- CAB1559
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Description
| Antibody Name: | Anti-PAH Antibody |
| Antibody SKU: | CAB1559 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB IF |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-290 of human PAH (NP_000268.1). |
| Application: | WB IF |
| Recommended Dilution: | WB 1:500 - 1:2000 IF 1:50 - 1:200 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | U-251MG, HepG2, Mouse liver, Mouse kidney |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-290 of human PAH (NP_000268.1). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | MSTA VLEN PGLG RKLS DFGQ ETSY IEDN CNQN GAIS LIFS LKEE VGAL AKVL RLFE ENDV NLTH IESR PSRL KKDE YEFF THLD KRSL PALT NIIK ILRH DIGA TVHE LSRD KKKD TVPW FPRT IQEL DRFA NQIL SYGA ELDA DHPG FKDP VYRA RRKQ FADI AYNY RHGQ PIPR VEYM EEEK KTWG TVFK TLKS LYKT HACY EYNH IFPL LEKY CGFH EDNI PQLE DVSQ FLQT CTGF RLRP VAGL LSSR DFLG GLAF RVFH CTQY IRHG SKPM YTPE PDIC HELL GH |
| Gene ID: | 5053 |
| Uniprot: | P00439 |
| Cellular Location: | |
| Calculated MW: | 51kDa |
| Observed MW: | 55kDa |
| Synonyms: | PAH, PH, PKU, PKU1 |
| Background: | 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. |
| 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 |
 | Western blot analysis of extracts of various cell lines, using PAH antibody (CAB1559) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. |
 | Immunofluorescence analysis of L929 cells using PAH antibody (CAB1559) at dilution of 1:100. Blue: DAPI for nuclear staining. |
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