Fumarase antibody

Principal name

Fumarase antibody

Alternative names for Fumarase antibody

HLRCC, LRCC, MCL, MCUL1, mitochondrial Fumarate hydratase

SwissProt ID

P07954 (Human), P10173 (Pig), P14408 (Rat), P97807 (Mouse)

Gene ID

2271 (FH)

Available reactivities

Hu (Human), Por (Porcine), Bov (Bovine), Can (Canine), Chk (Chicken), Eq (Equine), Fi (Fish), Hst (Hamster), Insect, Mky (Monkey), Ms (Mouse), Op (Opossum), Prim (Primate), Rt (Rat), Sh (Sheep), Xen (Xenopus), Tr (Turkey), L (Lizard), Bear

Available hosts

Mouse, Goat, Rabbit

Available applications

Enzyme Immunoassay (E), Immunocytochemistry/Immunofluorescence (ICC/IF), Western blot / Immunoblot (WB), Paraffin Sections (P), Dot blot (Dot), Immunodiffusion (ID), Immunoprecipitation (IP), Radioimmunoassay (R), Flow Cytometry (F)

Background of Fumarase antibody

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].

General readings

Lehtonen R., et al. (2003) J. Med. Genet. 40 (3): e19.
Toro JR., et al. (2003) Am. J. Hum. Genet. 73(1): 95-106.

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Primary Antibodies

Catalog No. Host Iso. Clone Pres. React. Applications  

Fumarase antibody

  FH (Fumarate Hydratase) mouse monoclonal antibody, clone OTI1F10 (formerly 1F10)  
Immunohistochemical staining of paraffin-embedded Human colon tissue within the normal limits using anti-FH mouse monoclonal antibody. (Heat-induced epitope retrieval by 10mM citric buffer, pH6.0, 100C for 10min, TA500675) Mouse IgG1 OTI1F10 Purified Can, Hu, Rt F, ICC/IF, P, WB
0.1 ml / €350.00
  OriGene Technologies, Inc.

TrueMAB

Fumarase antibody

  FH mouse monoclonal antibody, clone OTI9G4 (formerly 9G4)  
Immunohistochemical staining of paraffin-embedded Human breast tissue within the normal limits using anti-FH mouse monoclonal antibody. (Heat-induced epitope retrieval by 10mM citric buffer, pH6.0, 100C for 10min, TA500682) Mouse IgG1 OTI9G4 Purified Can, Hu, Rt F, ICC/IF, P, WB
0.1 ml / €379.00
  OriGene Technologies, Inc.

TrueMAB

2 Item(s)

per page
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