FAH Antibody

Code CSB-PA007965LA01HU
Size US$166
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  • Western Blot
    Positive WB detected in: Mouse kidney tissue, Mouse stomach tissue
    All lanes: FAH antibody at 3.4µg/ml
    Secondary
    Goat polyclonal to rabbit IgG at 1/50000 dilution
    Predicted band size: 47, 39 kDa
    Observed band size: 47 kDa

  • Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA007965LA01HU at dilution of 1:100

  • Immunofluorescent analysis of Hela cells using CSB-PA007965LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) FAH Polyclonal antibody
Uniprot No.
Target Names
FAH
Alternative Names
FAHFumarylacetoacetase antibody; FAA antibody; EC 3.7.1.2 antibody; Beta-diketonase antibody; Fumarylacetoacetate hydrolase antibody
Raised in
Rabbit
Species Reactivity
Human, Mouse
Immunogen
Recombinant Human Fumarylacetoacetase protein (2-250AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The FAH Antibody (Product code: CSB-PA007965LA01HU) is Non-conjugated. For FAH Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA007965LB01HU FAH Antibody, HRP conjugated ELISA
FITC CSB-PA007965LC01HU FAH Antibody, FITC conjugated
Biotin CSB-PA007965LD01HU FAH Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Form
Liquid
Tested Applications
ELISA, WB, IHC, IF
Recommended Dilution
Application Recommended Dilution
WB 1:2000-1:5000
IHC 1:20-1:200
IF 1:50-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Usage
For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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Target Background

Gene References into Functions
  1. Altogether these findings elucidate the molecular basis of HT1 caused by the frequent FAH c.1062+5G>A mutation, and demonstrate the compensatory effect of the c.1061C>A change in promoting exon definition, thus unraveling a rare mechanism leading to FAH immune-reactive mosaicism. PMID: 29497141
  2. molecular aspects of the FAH gene and its corresponding protein and a complete listing of all the mutations identified to date; highlight of the importance of splicing mutations in hereditary tyrosinemia type 1 PMID: 28755182
  3. Results from whole exome sequencing revealed a novel homozygous missense variant in FAH causing tyrosinemia type I . This novel variant involves the catalytic pocket of the enzyme, but does not result in increased succinylacetone or tyrosine. PMID: 27397503
  4. FAH gene mutation is associated with tyrosinemia type 1. PMID: 26565546
  5. Four splicing mutations affecting exonic or intronic nucleotides of the FAH gene were identified in two hereditary tyrosinemia type I patients. PMID: 23895425
  6. Two siblings have been described with tyrosinemia type 1 complicated by reversible hypertrophic cardiomyopathy in infancy due to a FAH homozygous mutation. PMID: 24016420
  7. Compound mutations (R237X and L375P) in the fumarylacetoacetate hydrolase gene causing tyrosinemia type I in a Chinese patient. PMID: 22884142
  8. Identification of novel mutations in the fumarylacetoacetase gene in Hereditary tyrosinaemia type I. PMID: 22554029
  9. We detected 11 novel and 6 previously described pathogenic mutations in the fumarylacetoacetase gene in a cohort of 43 patients originating from the Middle East with the acute form hereditary tyrosinemia type I PMID: 21764616
  10. A missense mutation in the fumarylacetoacetate hydrolase gene, responsible for hereditary tyrosinemia, acts as a splicing mutation. PMID: 11476670
  11. Data describe the metabolism of fumarylacetoacetate hydrolase mRNA harboring a nonsense mutation, W262X, in lymphoblastoid cell lines derived from hereditary tyrosinemia type I patients. PMID: 15465000
  12. identification of an alternative nonsense transcript of the fah gene, which despite being subjected to nonsense-mediated mRNA decay, produces a protein in different human tissues PMID: 15638932
  13. An immunopositive liver nodule was found in a patient with tyrosinemia having a mosaic pattern of FAH. PMID: 15759101

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Involvement in disease
Tyrosinemia 1 (TYRSN1)
Protein Families
FAH family
Tissue Specificity
Mainly expressed in liver and kidney. Lower levels are also detected in many other tissues.
Database Links

HGNC: 3579

OMIM: 276700

KEGG: hsa:2184

STRING: 9606.ENSP00000261755

UniGene: Hs.73875

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