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Rabbit Anti-FH Polyclonal Antibody#abs136972

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Description

Catalog-specification	Delivery time	USD price
abs136972-100ug	1-2 Weeks	301.0
abs136972-50ug	1-2 Weeks	201.0

Overview
catalog	abs136972
Description	This particular gene encodes a protein that serves as an essential component of the Krebs cycle, otherwise known as the tricarboxylic acid cycle. Its primary function is to catalyze the transformation of fumarate into L-malate. There are two distinct forms of this protein—one that resides within the cytosol and another that has an extended N-terminal and is directed towards the mitochondria. Interestingly, removal of the N-terminal leads to a molecule that appears identical to the cytoplasmic version. This protein functions as a homotetramer and shares many qualities with class II fumarases that are thermostable. When mutations occur in this gene, it can lead to a form of encephalopathy and ultimately fumarase deficiency.
Other names	Fumarase, also known as fumarate hydratase, is a mitochondrial enzyme coded by the FUMH_HUMAN gene. It is responsible for the conversion of fumarate to malate in the tricarboxylic acid cycle, a key process in cellular respiration. Mutations in the FUMH_HUMAN gene have been linked to various diseases, including hereditary leiomyomatosis and renal cell cancer (HLRCC), multiple hereditary cutaneous leiomyomata (MCL), and others. Fumarase plays a crucial role in ensuring optimal cellular function and energy production and is therefore of great interest to researchers and medical professionals alike.
Source	Rabbit
Specificity	FH Antibody detects endogenous levels of total FH.
Species Reactivity	Human;Mouse;Rat
Antigen	FH
Application	The recommended dilution for Western blotting ranges from 1:500 to 1:2000, while for Immunohistochemistry it is recommended to use a dilution range of 1:50 to 1:200. For ELISA (peptide), the recommended dilution is 1:20000 to 1:40000. These guidelines should be followed to ensure accurate and reliable results. Dilution beyond these recommended ranges may result in decreased sensitivity and specificity of the assay. It is important to note that the optimal dilution may vary depending on the specific experiment, and optimization of dilution may be necessary for optimal results.
Immunogen	A synthesized peptide derived from human FH.
MW	54kDa
Properties
Concentration	1mg/ml
purification	Using SulfoLink™ Coupling Resin, a peptide affinity chromatography process was employed to purify the antiserum.
Clonality	Polyclonal Antibody
Stability & Storage	Store at -20 Â°C for one year. Avoid repeated freeze/thaw cycles
Storage buffer	Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 Â°C.Stable for 12 months from date of receipt.
Target
Background	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.
Celluar localization	Extracellular region or secreted;Mitochondrion;
UniPort	P07954