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Anti-FH Antibody

品牌：Antibodies

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规格：100µg

货期：

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Anti-FH Antibody

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Name:	Anti-FH Antibody See all FH primary antibodies
Description:	Goat polyclonal antibody to FH.
Applications:	ELISA, WB, IHC, EIA
Reactivity:	Human
Immunogen:	Synthetic peptide corresponding to Human FH (internal region).
Sequence:	C-HPNDHVNKSQSSND
Host:	Goat
Clonality:	Polyclonal
Isotype:	IgG
Conjugate:	Unconjugated
Purification:	Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Concentration:	100 μg at 0.5 mg/ml.
Product Form:	Liquid
Formulation:	Supplied in Tris Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 0.5% BSA.
Storage:	Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

Function:	Also acts as a tumor suppressor.
Tissue Specificity:	Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology.
Involvement in Disease:	Fumarase deficiency: 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: A disorder characterized by predisposition to cutaneous and uterine leiomyomas, and papillary type 2 renal cancer which occurs in about 20% of patients.
Sequence Similarities:	Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.
Cellular Location:	Mitochondrion.
Database Links:	Entrez Gene: 2271?Human Omim: 136850?Human SwissProt: P07954?Human Unigene: 592490?Human
Synonyms:	FH Antibody Fumarase Antibody Fumarate hydratase Antibody Fumarate hydratase mitochondrial Antibody Fumarate hydratase, mitochondrial Antibody FUMH_HUMAN Antibody HLRCC Antibody LRCC Antibody MCL Antibody MCUL 1 Antibody MCUL1 Antibody MS709 Antibody Multiple hereditary cutaneous leiomyomata Antibody
Information:	Target information shown above is from the UniProt Consortium.