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FAH Antibody – Rabbit Polyclonal

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS6297661

This gene encodes the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT). More details

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Product Documentation
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Sample Manual Insert View PDF
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Testing Data View PDF
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Material Safety Data Sheet View PDF
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Technical Datasheet View PDF
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Product Overview

Online Datasheet in PDF
Product Name
FAH, Polyclonal Antibody
Full Product Name
FAH, NT (FAH, Fumarylacetoacetase, Beta-diketonase, Fumarylacetoacetate hydrolase) (FITC)
Product Gene Name
anti-FAH antibody
Product Synonym Names
FAH; Fumarylacetoacetase; Beta-diketonase; Fumarylacetoacetate hydrolase
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human
Form/Format
Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).
Purity/Purification
Purified by Protein A Affinity Chromatography.
Preparation and Storage
Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Molecular Weight
38,614 Da
OMIM
phenotype 613871
3D Structure
ModBase 3D Structure for P16930
Immunogen
FAH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 14-41 amino acids from the N-terminal region of human FAH.
Conjugate
FITC
Note
Preservative Free
Special Handling
Light sensitive

Product Categories/Family for anti-FAH antibody

Antibodies; Enzymes
Applications Tested/Suitable for anti-FAH antibody
Immunofluorescence (IF), FLISA, Immunohistochemistry (IHC), Western Blot (WB)
Application Notes for anti-FAH antibody
IF: 1:10-50
FLISA: 1:1,000
IHC: 1:50-100
WB: 1:100-500
Applications are based on unconjugated antibody.

Western Blot (WB) of anti-FAH antibody

FAH Antibody (N-term) western blot analysis in HepG2 cell line lysates (35ug/lane).This demonstrates the FAH antibody detected the FAH protein (arrow).
Rabbit Anti-FAH Polyclonal Antibody – Cat# MBS6297661

Immunohistochemistry (IHC) of anti-FAH antibody

FAH Antibody (N-term) immunohistochemistry analysis in formalin fixed and paraffin embedded human liver tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of FAH Antibody (N-term) for immunohistochemistry. Clinical relevance has not been evaluated.
Rabbit Anti-FAH Polyclonal Antibody, Immunohistochemistry (IHC) – Cat# MBS6297661

Immunofluorescence (IF) of anti-FAH antibody

Confocal immunofluorescent analysis of FAH Antibody (N-term) with HepG2 cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).
Rabbit Anti-FAH Polyclonal Antibody, Immunofluorescence (IF) – Cat# MBS6297661

Product References and Citations for anti-FAH antibody

Joslyn, G., et al. Alcohol. Clin. Exp. Res. 34(5):800-812(2010). Liu, J., et al. Hum Brain Mapp 30(1):241-255(2009). Ferreira, M.A., et al. Nat. Genet. 40(9):1056-1058(2008). Bliksrud, Y.T., et al. J. Mol. Med. 83(5):406-410(2005). Dreumont, N., et al. BMC Mol. Biol. 6 (1), 1 (2005).

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.

Searchable Terms for FAH purchase

MBS6297661 is an antibody targeting FAH. Also known as FAH, NT (FAH, Fumarylacetoacetase, Beta-diketonase, Fumarylacetoacetate hydrolase) (FITC). Reactive in Human. Host: Rabbit.

Additional Details

NCBI/Uniprot data below describe general gene information for FAH. It may not necessarily be applicable to this product.

NCBI GI #
4557587
NCBI GeneID
2184
NCBI Accession #
NP_000128
UniProt Primary Accession #
P16930
UniProt Secondary Accession #
Q53XA7; B2R9X1; D3DW95
UniProt Related Accession #
P16930
GenBank Nucleotide #
NM_000137
NCBI Official Full Name
fumarylacetoacetase
NCBI Official Synonym Full Names
fumarylacetoacetate hydrolase (fumarylacetoacetase)
NCBI Official Symbol
FAH
UniProt Protein Name
Fumarylacetoacetase
UniProt Synonym Protein Names
Beta-diketonase; Fumarylacetoacetate hydrolase
Protein Family
Fumarylacetoacetase
UniProt Gene Name
FAH
UniProt Synonym Gene Names
FAA
UniProt Entry Name
FAAA_HUMAN
NCBI Protein Information
fumarylacetoacetase; FAA; beta-diketonase

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
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Precautions

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

Disclaimer

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.

Product Note

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual, it is intended to serve as an example only. Please refer to the instructions For Use provided with the assay kit for precise details.

Product PubMed Publications

Products associated with anti-FAH antibody

 Reference Product  PubMed Publications
 HPD antibody  >8 publications with FAH and HPD
 GSTZ1 antibody  >1 publications with FAH and GSTZ1

Pathways associated with anti-FAH antibody

 Products by Pathway  Pathway Diagram
 Metabolic Pathways antibodies  Metabolic Pathways Diagram
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 Metabolism Of Amino Acids And Derivatives Pathway antibodies  Metabolism Of Amino Acids And Derivatives Pathway Diagram
 Phenylalanine And Tyrosine Catabolism Pathway antibodies  Phenylalanine And Tyrosine Catabolism Pathway Diagram
 Tyrosine Degradation, Tyrosine => Homogentisate Pathway antibodies  Tyrosine Degradation, Tyrosine => Homogentisate Pathway Diagram
 Tyrosine Degradation, Tyrosine => Homogentisate Pathway antibodies  Tyrosine Degradation, Tyrosine => Homogentisate Pathway Diagram
 Tyrosine Metabolism Pathway antibodies  Tyrosine Metabolism Pathway Diagram
 Tyrosine Metabolism Pathway antibodies  Tyrosine Metabolism Pathway Diagram
 Tyrosine Degradation Pathway antibodies  Tyrosine Degradation Pathway Diagram
 Tyrosine Degradation I Pathway antibodies  Tyrosine Degradation I Pathway Diagram

Diseases associated with anti-FAH antibody

 Disease Name  Pubmed Publications
 Liver Diseases Antibodies  >99 publications with FAH and Liver Diseases
 Brain Diseases Antibodies  >71 publications with FAH and Brain Diseases
 Tyrosinemias Antibodies  >64 publications with FAH and Tyrosinemias
 Disease Models, Animal Antibodies  >60 publications with FAH and Disease Models, Animal
 Liver Neoplasms Antibodies  >41 publications with FAH and Liver Neoplasms
 Carcinoma, Hepatocellular Antibodies  >38 publications with FAH and Carcinoma, Hepatocellular
 Neoplasms, Experimental Antibodies  >21 publications with FAH and Neoplasms, Experimental
 Liver Neoplasms, Experimental Antibodies  >20 publications with FAH and Liver Neoplasms, Experimental
 Precancerous Conditions Antibodies  >18 publications with FAH and Precancerous Conditions
 Adenocarcinoma Antibodies  >16 publications with FAH and Adenocarcinoma

Organs/Tissues associated with anti-FAH antibody

 Organ/Tissue Name  Pubmed Publications
 Liver Antibodies  >187 publications with FAH and Liver
 Blood Antibodies  >103 publications with FAH and Blood
 Kidney Antibodies  >47 publications with FAH and Kidney
 Bone Antibodies  >26 publications with FAH and Bone
 Skin Antibodies  >24 publications with FAH and Skin
 Brain Antibodies  >13 publications with FAH and Brain
 Lung Antibodies  >11 publications with FAH and Lung
 Muscle Antibodies  >11 publications with FAH and Muscle
 Embryonic Tissue Antibodies  >8 publications with FAH and Embryonic Tissue
 Intestine Antibodies  >8 publications with FAH and Intestine
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