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Rabbit Anti-FH Polyclonal Antibody

Cat NoBSPA-1866
Conjugate
TypePolyclonal Antibody
SourceRabbit
Size1 mg
ApplicationELISA; WB; IHC
FormatLiquid
ConcentrationPlease refer to the vial lable for the specific concentration.
BufferSupplied in PBS.
SpeciesHuman
StorageStore at -20 degree. Avoid repeted freeze/thaw cycl.
SynonymsFH;fumarate hydratase;MCL;LRCC;HLRCC;MCUL1;fumarate hydratase, mitochondrial;fumarase
Purification
NoteThis product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
MolecularWeight
Description
BackgroundThe protein encoded by this gene is an enzymatic component of the tricarboxylic acid cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy.
We can offer labeled proteins/antibodies using a broad range of intensely fluorescent dyes and labels including FITC, Biotin, Alexa Fluor, Rhodamine B, Cy, Co-Au.