Product Name	Fumarylacetoacetate hydrolase
Chinese Name	延胡索酰乙酰乙酸水解酶抗体
Alias	Beta diketonase; FAA; FAAA_HUMAN; FAH; Fumarylacetoacetase; Fumarylacetoacetate; Fumarylacetoacetate hydrolase.
literatures	Specific References  (1)     |     SL16194R has been referenced in 1 publications. [IF=2.634] Gao M et al. Efficient Generation of an Fah/Rag2 Dual-Gene Knockout Porcine Cell Line Using CRISPR/Cas9and Adenovirus. DNA Cell Biol. 2019 Feb 14.  ICC&IF ;  Porcine.   PubMed:30762444
Research Area	Tumour  Cell biology  immunology  Cytoskeleton  The new supersedes the old
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rat, Pig, )
Applications	ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	47kDa
Cellular localization	cytoplasmic Extracellular matrix Secretory protein
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human Fumarylacetoacetate hydrolase: 21-120/419
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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] Function: Fumarylacetoacetate hydrolase is the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT). This is an autosomal recessive inborn error of metabolism that occurs in both an acute and a chronic form. Clinical characteristics of the acute form include hepatic failure and death in infancy, whereas children with the chronic form have renal tubular dysfunction and hypophosphatemic rickets, progressive liver disease with 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. Subunit: Homodimer. Tissue Specificity: Mainly expressed in liver and kidney. Lower levels are also detected in many other tissues. Similarity: Belongs to the FAH family. SWISS: P16930 Gene ID: 2184 Database links: Entrez Gene: 2184 Human Entrez Gene: 14085 Mouse Entrez Gene: 29383 Rat Omim: 613871 Human SwissProt: P16930 Human SwissProt: P35505 Mouse SwissProt: P25093 Rat

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