Anti-PAH Antibody (CAB1559)

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SKU:
CAB1559
Product type:
Antibody
Reactivity:
Human
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism
Frequently bought together:

Description

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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
Anti-PAH Antibody (CAB1559)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.
Anti-PAH Antibody (CAB1559)Immunofluorescence analysis of L929 cells using PAH antibody (CAB1559) at dilution of 1:100. Blue: DAPI for nuclear staining.

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