FH Antibody
Description
Fumarate Hydratase (FH) Antibodies
Fumarate Hydratase (FH) is a mitochondrial enzyme catalyzing the reversible hydration of fumarate to malate in the TCA cycle. Defects in FH are linked to hereditary leiomyomatosis and renal cell carcinoma (HLRCC), as well as fumaric aciduria. Antibodies targeting FH are primarily used in research to study its expression, subcellular localization, and pathological roles.
Research Insights
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Subcellular Localization: FH antibodies confirm cytoplasmic and mitochondrial localization of the enzyme .
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Pathological Relevance: Defects in FH lead to fumaric aciduria, characterized by encephalopathy and metabolic acidosis .
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Cancer Research: Overexpression of FH is studied in HLRCC, where antibodies aid in diagnosing tumor-associated mutations .
Factor H (FH) Antibodies
Factor H (FH) is a complement regulator that binds C3b, preventing excessive complement activation. Autoantibodies against FH are implicated in atypical hemolytic uremic syndrome (aHUS), a disorder causing thrombocytopenia, hemolytic anemia, and renal failure.
Role in Disease and Therapeutic Targets
Mechanistic Insights
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Complement Regulation: FH stabilizes C3bBb convertase decay and recruits factor I for C3b cleavage .
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Genetic Predisposition: Anti-FH antibodies are linked to CFHR1/CFHR3 deletions, though not all carriers develop aHUS .
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Treatment Outcomes: Plasma exchange and immunosuppression reduce mortality and improve renal function in aHUS patients .
Comparative Analysis of FH Antibodies
| Feature | Fumarate Hydratase (FH) Antibodies | Factor H (FH) Antibodies |
|---|---|---|
| Primary Use | Research (WB, IHC, IF) | Diagnostic/Clinical (aHUS) |
| Host/Conjugate | Rabbit/Goat/Mouse; Cy5/HRP/FITC | Human (autoantibodies) or engineered mAbs |
| Key Targets | Mitochondrial/cytoplasmic FH | C3b-binding domains of FH |
| Clinical Impact | Diagnostic for HLRCC, fumaric aciduria | Prognostic marker for aHUS severity |
Future Directions
Product Specs
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Target Background
- Frataxin deficiency disrupts the normal functioning of chloroplasts by impacting the levels of iron, chlorophyll, and the photosynthetic electron transport chain within this organelle. PMID: 26517126
- AtFH, beyond its role in safeguarding bioavailable iron within mitochondria and the biogenesis of Fe-S groups, also participates in the biosynthesis of heme groups in plants. PMID: 21166997
- These findings indicate that frataxin is an essential protein in plants, required for full activity of mitochondrial Fe-S proteins and playing a protective role against oxidative damage. [frataxin] [ATFH] PMID: 17092311
- Findings suggest a key role of plant frataxin during embryogenesis. PMID: 17258206
- Frataxin deficiency also leads to nitric oxide (NO) accumulation in both atfh-1 roots and frataxin null mutant yeast. PMID: 19114041
