Mouse Anti-FH Recombinant Antibody (VS3-CJ51) (CAT#: VS3-CJ51)

• Immunogen
• Recombinant fragment of human FH (C-terminus)

• Host Species
• Mouse

• Type
• Mouse IgG

• Specificity
• Human FH

• Species Reactivity
• Human

• Applications
• WB, ELISA

• Conjugate
• Unconjugated

• Purification
• Protein G affinity purified

• Purity
• >95% as determined by SDS-PAGE

• Format
• Liquid

• Concentration
• 1 mg/mL (lot specific)

• Buffer
• 50% Glycerol, 1% BSA, PBS, pH7.4.

• Preservative
• 0.02% sodium azide

• Storage
• Store at 4°C for short term. Aliquot and store at -20°C for long term. Avoid repeated freeze/thaw cycles.

• Shipping
• Ice packs

• Application Notes
• This antibody has been tested for use in ELISA (1:5000-1:20000), Western Blot (1:100-1:2000).

• Alternative Names
• FMRD; HLRCC; HsFH; LRCC; MCL; MCUL1

• Gene ID
• 2271

• UniProt ID
• P07954

• Sequence Similarities
• Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.

• Cellular Localization
• Chromosome, Mitochondrion, Cytoplasm, Nucleus

• Post Translation Modifications
• Isoform Cytoplasmic:
  Phosphorylation at Thr-236 by PRKDC in response to DNA damage promotes translocation to the nucleus and recruitment to DNA double-strand breaks (DSBs).

• Protein Refseq
• NP_000134.2

• Function
• Catalyzes the reversible stereospecific interconversion of fumarate to L-malate (PubMed:30761759).
  Experiments in other species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable).
  Isoform Mitochondrial:
  Catalyzes the hydration of fumarate to L-malate in the tricarboxylic acid (TCA) cycle to facilitate a transition step in the production of energy in the form of NADH.
  Isoform Cytoplasmic:
  Catalyzes the dehydration of L-malate to fumarate (By similarity).
  Fumarate metabolism in the cytosol plays a role during urea cycle and arginine metabolism; fumarate being a by-product of the urea cycle and amino-acid catabolism (By similarity).
  Also plays a role in DNA repair by promoting non-homologous end-joining (NHEJ) (PubMed:20231875, PubMed:26237645).
  In response to DNA damage and phosphorylation by PRKDC, translocates to the nucleus and accumulates at DNA double-strand breaks (DSBs): acts by catalyzing formation of fumarate, an inhibitor of KDM2B histone demethylase activity, resulting in enhanced dimethylation of histone H3 'Lys-36' (H3K36me2) (PubMed:26237645).

Isotype Control

• MRO-A1591-YJ
• Mouse IgG Isotype control, Polyclonal

Secondary Antibody

• 2ND-040CQ
• Goat Anti-Mouse IgG (H&L) Antibody

• 2ND-042CQ
• Rabbit Anti-Mouse IgG (H&L) Antibody

Recommended Dilution Buffer

• MRO-0253YJ
• Reconstitution Buffer PBS

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