FH antibody
Quick Overview for FH antibody (ABIN568200)
Target
See all FH AntibodiesReactivity
Host
Clonality
Conjugate
Application
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Purification
- Ammonium Sulphate Precipitation and Ion Exchange Chromatography
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Immunogen
- Fumarase is isolated and purified from Porcine heart Freund’s complete adjuvant is used in the first step of the immunization procedure.
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Isotype
- IgG
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Application Notes
- Optimal working dilution should be determined by the investigator.
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Restrictions
- For Research Use only
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Reconstitution
- Restore by adding 1.0 mL of sterile distilled water
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Concentration
- 10.0 mg/mL
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Buffer
- PBS, pH 7.2 without preservatives and foreign proteins
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Preservative
- Without preservative
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Storage
- 4 °C/-20 °C
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Storage Comment
- Store the antibody lyophilized at 2-8 °C and reconstituted at 2-8 °C for one week or (in aliquots) at -20 °C for longer. If a slight precipitation occurs upon storage, this should be removed by centrifugation.
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- FH (Fumarate Hydratase (FH))
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Alternative Name
- Fumarase
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Background
- 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].Synonyms: HLRCC, LRCC, MCL, MCUL1, mitochondrial Fumarate hydratase
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Gene ID
- 9823
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UniProt
- P10173
Target
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