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F9180

Sigma-Aldrich

Anti-FH (61-75) antibody produced in rabbit

IgG fraction of antiserum, buffered aqueous solution

Synonym(s):

Anti-Fumarate hydratase precursor, Anti-HLRCC, Anti-LRCC, Anti-MCL, Anti-MCUL1

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About This Item

UNSPSC Code:
12352203

biological source

rabbit

conjugate

unconjugated

antibody form

IgG fraction of antiserum

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

antigen ~55 kDa

species reactivity

human

technique(s)

western blot: 1:500-1:2,000

UniProt accession no.

shipped in

dry ice

storage temp.

−20°C

Gene Information

human ... FH(2271)

Immunogen

synthetic peptide corresponding to amino acids 61-75 of human FH.

Application

Anti-FH (61-75) antibody is suitable for western blot at a dilution of 1:500-1:2,000.
Yale Center for High Throughput Cell Biology IF-tested antibodies. Each antibody is tested by immunofluorescence against HUVEC cells using the Yale HTCB IF protocol. To learn more about us and Yale Center for High Throughput Cell Biology partnership, visit sigma.com/htcb-if.

Biochem/physiol Actions

FH gene is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle and catalyzes the formation of L-malate from fumarate. It is expressed in red blood cells but under expressed in red blood cells (cytoplasm) of patients suffering from hereditary non-spherocytic hemolytic anemia. It plays a vital role in adenosine triphosphate (ATP) production through the mitochondrial respiratory chain. Deficiency of fumarate hydratase causes a rare autosomal recessive disorder of the citric acid cycle which followed by severe neurological impairment.

Physical form

Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.

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Storage Class

12 - Non Combustible Liquids

wgk_germany

WGK 2

flash_point_f

Not applicable

flash_point_c

Not applicable

ppe

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)


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Patrick J Pollard et al.
Annals of medicine, 35(8), 632-639 (2004-01-08)
It is well documented that disturbances in mitochondrial function are associated with rare childhood disorders and possibly with many common diseases of ageing, such as Parkinson's disease and dementia. There has also been increasing evidence linking mitochondrial dysfunction with tumorigenesis.
Ian P M Tomlinson et al.
Nature genetics, 30(4), 406-410 (2002-02-28)
Uterine leiomyomata (fibroids) are common and clinically important tumors, but little is known about their etiology and pathogenesis. We previously mapped a gene that predisposes to multiple fibroids, cutaneous leiomyomata and renal cell carcinoma to chromosome 1q42.3-q43 (refs 4-6). Here
E M Coughlin et al.
Molecular genetics and metabolism, 63(4), 254-262 (1998-06-23)
Fumarase deficiency is a rare autosomal recessive disorder of the citric acid cycle causing severe neurological impairment. The cDNA for both the rat and human enzymes has been cloned previously and shown to encode a coding region of 1.46 kb.
Katharina von Löhneysen et al.
PloS one, 7(4), e34237-e34237 (2012-04-18)
Erythrocyte cytosolic protein expression profiles of children with unexplained hemolytic anemia were compared with profiles of close relatives and controls by two-dimensional differential in-gel electrophoresis (2D-DIGE). The severity of anemia in the patients varied from compensated (i.e., no medical intervention

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