Full Product Name
FAH antibody - C-terminal region
Product Gene Name
anti-FAH antibody
[Similar Products]
Antibody/Peptide Pairs
FAH peptide (MBS3230937) is used for blocking the activity of FAH antibody (MBS3205975)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Immunogen Sequence
Synthetic peptide located within the following region: AATICKSNFK YMYWTMLQQL THHSVNGCNL RPGDLLASGT ISGPEPENFG
3D Structure
ModBase 3D Structure for P16930
Species Reactivity
Cow, Dog, Guinea Pig, Horse, Human, Mouse, Rabbit, Rat, Zebrafish
Purity/Purification
Protein A purified
Form/Format
Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Homology
Cow: 93%; Dog: 100%; Guinea Pig: 93%; Horse: 100%; Human: 100%; Mouse: 100%; Rabbit: 93%; Rat: 100%; Zebrafish: 93%
Immunogen
The immunogen is a synthetic peptide directed towards the C terminal region of human FAH
Preparation and Storage
For short term use, store at 2-8 degree C up to 1 week. For long term storage, store at -20 degree C in small aliquots to prevent freeze-thaw cycles.
Other Notes
Small volumes of anti-FAH antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-FAH antibody
This is a rabbit polyclonal antibody against FAH. It was validated on Western Blot and immunohistochemistry
Target Description: FAH is the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia.This gene encodes the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT).
Product Categories/Family for anti-FAH antibody
Polyclonal; Signal Proteins; Lymphocyte Signaling; Membrane Protein; Drugs and Drug Metabolism; Developmental Biology; Immunohistochemistry; Receptors;
Applications Tested/Suitable for anti-FAH antibody
Immunohistochemistry (IHC), Western Blot (WB)
Immunohistochemistry (IHC) of anti-FAH antibody
Immunohistochemistry with human liver, mouse KO tissue
Immunohistochemistry (IHC) of anti-FAH antibody
Immunohistochemistry with human liver, mouse KO tissue
Immunohistochemistry (IHC) of anti-FAH antibody
Rabbit Anti-FAH Antibody
Paraffin Embedded Tissue: Human Kidney
Cellular Data: Epithelial cells of renal tubule
Antibody Concentration: 4.0-8.0 ug/ml
Magnification: 400X
Immunohistochemistry (IHC) of anti-FAH antibody
Sample Type: Human Liver and Mouse FAH KO liverPrimary Dilution: 1:400
Western Blot (WB) of anti-FAH antibody
FAH antibody - C-terminal region validated by WB using Jurkat cell lysate at 2.5 ug/ml.
Western Blot (WB) of anti-FAH antibody
Host: Mouse
Target Name: FAH
Sample Tissue: Mouse Testis
Antibody Dilution: 1ug/ml
NCBI/Uniprot data below describe general gene information for FAH. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000128
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NCBI GenBank Nucleotide #
NM_000137
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UniProt Primary Accession #
P16930
[Other Products]
UniProt Related Accession #
P16930[Other Products]
NCBI Official Full Name
fumarylacetoacetase
NCBI Official Synonym Full Names
fumarylacetoacetate hydrolase
NCBI Official Symbol
FAH [Similar Products]
NCBI Protein Information
fumarylacetoacetase
UniProt Protein Name
Fumarylacetoacetase
UniProt Synonym Protein Names
Beta-diketonase; Fumarylacetoacetate hydrolase
Protein Family
Fumarylacetoacetase
UniProt Gene Name
FAH [Similar Products]
UniProt Synonym Gene Names
FAA [Similar Products]
UniProt Entry Name
FAAA_HUMAN
NCBI Summary for FAH
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 Comments for FAH
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.
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
Research Articles on FAH
1. 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.">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.
Precautions
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