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Anti-FAH Antibody (CAB13492)

SKU:
CAB13492
Product Type:
Antibody
Reactivity:
Human
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism
  • Metabolism Antibodies 1 Anti-FAH Antibody CAB13492
  • Metabolism Antibodies 1 Anti-FAH Antibody CAB13492
  • Metabolism Antibodies 1 Anti-FAH Antibody CAB13492
  • Metabolism Antibodies 1 Anti-FAH Antibody CAB13492
  • Metabolism Antibodies 1 Anti-FAH Antibody CAB13492
€139 - €419
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Description

system_update_altDatasheetsystem_update_altMSDS
Antibody Name:Anti-FAH Antibody
Antibody SKU:CAB13492
Antibody Size:20uL, 50uL, 100uL
Application:WB IHC
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-419 of human FAH (NP_000128.1).
Application:WB IHC
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:Mouse liver, Mouse kidney, Rat liver, Rat kidney
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-419 of human FAH (NP_000128.1).
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:MSFI PVAE DSDF PIHN LPYG VFST RGDP RPRI GVAI GDQI LDLS IIKH LFTG PVLS KHQD VFNQ PTLN SFMG LGQA AWKE ARVF LQNL LSVS QARL RDDT ELRK CAFI SQAS ATMH LPAT IGDY TDFY SSRQ HATN VGIM FRDK ENAL MPNW LHLP VGYH GRAS SVVV SGTP IRRP MGQM KPDD SKPP VYGA CKLL DMEL EMAF FVGP GNRL GEPI PISK AHEH IFGM VLMN DWSA RDIQ KWEY VPLG PFLG KSFG TTVS PWVV PMDA LMPF AVPN PKQD PRPL PYLC HDEP YTFD INLS VNLK GEGM SQAA TICK SNFK YMYW TMLQ QLTH HSVN GCNL RPGD LLAS GTIS GPEP ENFG SMLE LSWK GTKP IDLG NGQT RKFL LDGD EVII TGYC QGDG YRIG FGQC AGKV LPAL LPS
Gene ID:2184
Uniprot:P16930
Cellular Location:
Calculated MW:38kDa/46kDa
Observed MW:46kDa
Synonyms:FAH
Background:This gene encodes the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT).
UniProt Protein Function:FAH: Defects in FAH are the cause of tyrosinemia type 1 (TYRO1). An inborn error of metabolism characterized by elevations of tyrosine in the blood and urine, and hepatorenal manifestations. Typical features include hepatic necrosis, renal tubular injury, episodic weakness, self-mutilation, and seizures. Renal tubular dysfunction is associated with phosphate loss and hypophosphataemic rickets. Progressive liver disease can lead to the development of hepatocellular carcinoma. Dietary treatment with restriction of tyrosine and phenylalanine alleviates the rickets, but liver transplantation has so far been the only definite treatment. Belongs to the FAH family.
UniProt Protein Details:

Protein type:Amino Acid Metabolism - tyrosine; EC 3.7.1.2; Hydrolase

Chromosomal Location of Human Ortholog: 15q25.1

Cellular Component: cytosol

Molecular Function:fumarylacetoacetase activity; metal ion binding

Biological Process: L-phenylalanine catabolic process; tyrosine catabolic process; arginine catabolic process

Disease: Tyrosinemia, Type I

NCBI Summary:This gene encodes the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT). [provided by RefSeq, Jul 2008]
UniProt Code:P16930
NCBI GenInfo Identifier:119778
NCBI Gene ID:2184
NCBI Accession:P16930.2
UniProt Related Accession:P16930
Molecular Weight:~ 46kDa
NCBI Full Name:Fumarylacetoacetase
NCBI Synonym Full Names:fumarylacetoacetate hydrolase
NCBI Official Symbol:FAH  
NCBI Protein Information:fumarylacetoacetase
UniProt Protein Name:Fumarylacetoacetase
UniProt Synonym Protein Names:Beta-diketonase; Fumarylacetoacetate hydrolase
Protein Family:Fumarylacetoacetase
UniProt Gene Name:FAH  
UniProt Entry Name:FAAA_HUMAN
Anti-FAH Antibody (CAB13492)Western blot analysis of extracts of various cell lines, using FAH antibody (CAB13492) 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. Detection: ECL Basic Kit (RM00020). Exposure time: 5s.
Anti-FAH Antibody (CAB13492)Immunohistochemistry of paraffin-embedded rat liver using FAH antibody (CAB13492) at dilution of 1:100 (40x lens).
Anti-FAH Antibody (CAB13492)Immunohistochemistry of paraffin-embedded rat kidney using FAH antibody (CAB13492) at dilution of 1:100 (40x lens).
Anti-FAH Antibody (CAB13492)Immunohistochemistry of paraffin-embedded human liver damage using FAH antibody (CAB13492) at dilution of 1:100 (40x lens).
Anti-FAH Antibody (CAB13492)Immunohistochemistry of paraffin-embedded mouse liver using FAH antibody (CAB13492) at dilution of 1:100 (40x lens).

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