Product Name	Anti-FH/RBITC
Chinese Name	罗丹明（RBITC）标记的富马酸水合酶抗体
Alias	mitochondrial; Fumarase; Fumarate hydratase; Fumarate hydratase mitochondrial; FUMH_HUMAN; HLRCC; LRCC; MCL; MCUL 1; MCUL1; Multiple hereditary cutaneous leiomyomata.
Research Area	Tumour  Cell biology  immunology  transcriptional regulatory factor  Mitochondrion
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
Applications	IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	51kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human Fumarate hydratase
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Product Detail	background: The protein encoded by this gene is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy. [provided by RefSeq, Jul 2008] Function: Also acts as a tumor suppressor. Subunit: Homotetramer. Subcellular Location: Cytoplasm, Mitochondrion Tissue Specificity: Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology. DISEASE: Fumarase deficiency (FMRD): The disease is caused by mutations affecting the gene represented in this entry. A severe autosomal recessive metabolic disorder characterized by early-onset hypotonia, profound psychomotor retardation, and brain abnormalities, such as agenesis of the corpus callosum, gyral defects, and ventriculomegaly. Many patients show neonatal distress, metabolic acidosis, and/or encephalopathy. Hereditary leiomyomatosis and renal cell cancer (HLRCC): The disease is caused by mutations affecting the gene represented in this entry. A disorder characterized by predisposition to cutaneous and uterine leiomyomas, and papillary type 2 renal cancer which occurs in about 20% of patients. Similarity: Belongs to the class-II fumarase/aspartase family. Fumarase subfamily. Database links: Entrez Gene: 520260 Cow Entrez Gene: 480092 Dog Entrez Gene: 2271 Human Entrez Gene: 14194 Mouse Entrez Gene: 100627128 Pig Entrez Gene: 24368 Rat Entrez Gene: 393938 Zebrafish Omim: 136850 Human SwissProt: P07954 Human SwissProt: P97807 Mouse SwissProt: P10173 Pig SwissProt: P14408 Rat SwissProt: Q7SX99 Zebrafish Unigene: 592490 Human Unigene: 41502 Mouse Unigene: 29782 Rat Unigene: 104452 Zebrafish Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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