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Anti-FH Antibody

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产品名称: Anti-FH Antibody
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简单介绍

Anti-FH Antibody


Anti-FH Antibody  的详细介绍
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.
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