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Overview

Product name

Anti-FH antibody
See all FH products (7) ...

Description

Goat polyclonal to FH

Tested applications

WB, ELISAmore details

Cross reactivity

Reacts with

Human

Predicted to work with

Mouse, Rat, Horse, Cow, Pig, Fruit fly (Drosophila melanogaster), Zebrafish, a wide range of other species, Cynomolgus Monkey, Chlamydia trachomatis, Orangutan

Immunogen

Synthetic peptide: C-HPNDHVNKSQSSND, corresponding to internal sequence amino acids 176-189 of Human FH

C-HPNDHVNK SQSSND

Positive control

Human Kidney, Liver and Lung lysates

Properties

Form

Liquid

Storage instructions

Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

Storage buffer

Preservative: 0.02% Sodium Azide
Constituents: 0.5% BSA, Tris buffered saline, pH 7.3

Concentration

Concentration information loading...

Purity

Immunogen affinity purified

Purification notes

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.

Clonality

Polyclonal

Isotype

IgG

  • Western blot - FH antibody (ab62825)Western blot - FH antibody (ab62825) image (enlarge)

Applications

Show applications key

Our Abpromise guarantee covers the use of ab62825 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • ShowHide1 Image

    WB

     WB: Use a concentration ...Read more →

    WB: Use a concentration of 0.01 - 0.03 µg/ml.Detects a band of approximately 48 kDa (predicted molecular weight: 55 kDa).

  • ELISA

     ELISA: 1/64000

    ELISA: 1/64000

Target

Function

Also acts as a tumor suppressor.

Pathway

Carbohydrate metabolism; tricarboxylic acid cycle; (S)-malate from fumarate: step 1/1.

Involvement in disease

Defects in FH are the cause of fumarase deficiency (FHD) [MIM:606812]; also known as fumaricaciduria. FHD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia.
Defects in FH are the cause of multiple cutaneous and uterine leiomyomata (MCUL1) [MIM:150800]. MCUL1 is an autosomal dominant condition in which affected individuals develop benign smooth muscle tumors (leiomyomata) of the skin. Affected females also usually develop leiomyomata of the uterus (fibroids).
Defects in FH are the cause of hereditary leiomyomatosis and renal cell cancer (HLRCC) [MIM:605839].

Sequence similarities

Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.

Cellular localization

Cytoplasm and Mitochondrion.

Target information above from: UniProt accessionP07954 The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).

Information by UniProt

Alternative names

  • FH antibody
  • Fumarase antibody
  • Fumarate hydratase mitochondrial antibody
  • Fumarate hydratase, mitochondrial antibody
  • FUMH_HUMAN antibody
  • HLRCC antibody
  • LRCC antibody
  • MCL antibody
  • MCUL 1 antibody
  • MCUL1 antibody
  • MS709 antibody
  • Multiple hereditary cutaneous leiomyomata antibody
see all

Anti-FH antibody images:

  Western blot - FH antibody (ab62825)

Western blot - FH antibody (ab62825)

Anti-FH antibody (ab62825) at 0.01 µg/ml + Human Kidney lysate in RIPA buffer at 35 µg/ml

Predicted band size : 55 kDa
Observed band size : 48 kDa (why is the actual band size different from the predicted?)


Detected by chemiluminescence

References for Anti-FH antibody (ab62825)

This product has been referenced in:

  • Kienhöfer Jet al. Association of mitochondrial antioxidant enzymes with mitochondrial DNA as integral nucleoid constituents. FASEB J 23:2034-44 (2009). WB; Human.Read more (PubMed: 19228881) »

See 1 publication for this product

Publishing research using ab62825? Please let us know so that we can cite the reference in this datasheet

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"