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FH protein (Human) (ab82790)

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Overview

Product name

FH protein (Human)
See all FH products (2) ...

Protein description

Recombinant full length protein (Human) mature form of FH (without mitochondrion transit peptide), aa 44-510, MW 50.2 kDa, Swiss-Prot P07954.

Expression host

E. coli

Properties

Purity

>95% by SDS-PAGE

Form

Liquid

Storage instructions

Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

Storage buffer

Preservative: None
Constituents: 20mM Tris HCl, pH 8

Concentration

Concentration information loading...

Additional notes

Endotoxin Level: < 1.0 EU per 1µg of protein (determined by LAL method)

Sequence notes

MASQNSFRIE YDTFGELKVP NDKYYGAQTV RSTMNFKIGG VTERMPTPVI KAFGILKRAA AEVNQDYGLD PKIANAIMKA ADEVAEGKLN DHFPLVVWQT GSGTQTNMNV NEVISNRAIE MLGGELGSKI PVHPNDHVNK SQSSNDTFPT AMHIAAAIEV HEVLLPGLQK LHDALDAKSK EFAQIIKIGR THTQDAVPLT LGQEFSGYVQ QVKYAMTRIK AAMPRIYELA AGGTAVGTGL NTRIGFAEKV AAKVAALTGL PFVTAPNKFE ALAAHDALVE LSGAMNTTAC SLMKIANDIR FLGSGPRSGL GELILPENEP GSSIMPGKVN PTQCEAMTMV AAQVMGNHVA VTVGGSNGHF ELNVFKPMMI KNVLHSARLL GDASVSFTEN CVVGIQANTE RINKLMNESL MLVTALNPHI GYDKAAKIAK TAHKNGSTLK ETAIELGYLT AEQFDEWVKP KDMLGPK

Research areas

Cancer >> Cancer Metabolism >> Metabolic signaling pathway >> Integration of energy metabolism
Cancer >> Oncoproteins/suppressors >> Tumor suppressors >> Other
Signal Transduction >> Metabolism >> Energy Metabolism

  • SDS-PAGE - FH protein (Human) (ab82790)SDS-PAGE - FH protein (Human) (ab82790) image (enlarge)

Applications

Show applications key

Our Abpromise guarantee covers the use of ab82790 in the following tested applications.

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

Protein info

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

FH protein (Human) images:

  SDS-PAGE - FH protein (Human) (ab82790)

SDS-PAGE - FH protein (Human) (ab82790)

ab82790 on 15% SDS-PAGE (3µg)

References for FH protein (Human) (ab82790)

ab82790 has not yet been referenced specifically in any publications.

Publishing research using ab82790? 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"