Recombinant full length protein, corresponding to amino acids 33-511 of Human FH
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Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 55 kDa.
Use at an assay dependent concentration. PubMed: 22867999
Also acts as a tumor suppressor.
Carbohydrate metabolism; tricarboxylic acid cycle; (S)-malate from fumarate: step 1/1.
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].
Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.
Predicted band size : 55 kDa FH antibody (ab58232) at 1ug/lane + HeLa cell lysate at 25ug/lane.
Immunocytochemistry/ Immunofluorescence - Anti-FH antibody (ab58232)Image from Yang Y et al., Cancer Genetics, Volume 205, Issues 7–8, July–August 2012, Pages 377–390. Fig 1.; doi: 10.1016/j.cancergen.2012.05.001. with permission from Elsevier.
Immunofluorescence analysis of UOK268 cells, staining FH with ab58232.
Cells were fixed in formaldehyde and permeabilized with 0.2% Triton X-100. Samples were incubated with primary antibody (1/200) for 1 hour at room temperature. An AlexaFluor®488-conjugated goat anti-mouse IgG (1/500) was used as the secondary antibody.
Perrier-Trudova V et al. Fumarate Hydratase-deficient Cell Line NCCFH1 as a New In Vitro Model of Hereditary Papillary Renal Cell Carcinoma Type 2. Anticancer Res35:6639-53 (2015).
Read more (PubMed: 26637880) »
Beernink PT et al. Heterogeneity in rhesus macaque complement factor H binding to meningococcal factor H binding protein (FHbp) informs selection of primates to assess immunogenicity of FHbp-based vaccines. Clin Vaccine Immunol21:1505-11 (2014).
Read more (PubMed: 25185576) »