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|Sample type||Average %||Range|
|Serum||111||107% - 116%|
|Cell culture media||= 120||119% - 127%|
|Extraction Buffer||119||119% - 124%|
Does not react withMouse, Rat
Abcam’s Fumarase (FH) Human ELISA (Enzyme-Linked Immunosorbent Assay) kit is designed for the accurate quantitative measurement of Human Fumarase protein in Human cell and tissue lysates.
The assay employs an antibody specific to FH protein coated onto well plate strips. Standards and samples are pipetted into the wells and FH present in the sample is bound to the wells by the immobilized antibody. The wells are washed and an anti-FH detector antibody is added. After washing away unbound detector antibody, HRP-conjugated label specific for the detector antibody is pipetted into the wells. The wells are again washed, a TMB substrate solution is added to the wells and blue color develops in proportion to the amount of FH bound to the plate well. The developing blue color is measured at 600 nm. Optionally the reaction can be stopped by adding hydrochloric acid which changes the color from blue to yellow and the intensity can be measured at 450 nm.
Fumarase (FH, Fumarate hydratase, P07954) is a 50 kDa enzyme that catalyzes the reversible hydration/dehydration of fumarate to malate (EC 126.96.36.199). Fumarase comes in two forms: mitochondrial and cytosolic. The mitochondrial isoenzyme is involved in the Krebs Cycle (also known as the Tricarboxylic Acid Cycle [TCA] or the Citric Acid Cycle), and the cytosolic isoenzyme, exists without the mitochondrial import sequence, is involved in the metabolism of cytoplasmic fumarate, a byproduct of the urea and amino acid metabolism.
Defects in fumarase (FH) are the cause of fumarase deficiency [MIM606812], also known as fumaricaciduria, which is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic/pyruvic academia. Additionally FH is a tumor suppressor gene; inactivating mutations in the FH gene are responsible for hereditary leiomyomataosis renal cell carcinoma (HLRCC) [MIM:150800], a disorder characterized by predisposition to cutaneous and uterine leiomyomas, and papillary type 2 renal cancer which occurs in about 20% of patients.
|Components||1 x 96 tests|
|10X Blocking Buffer||1 x 6ml|
|10X FH Detector Antibody||1 x 1ml|
|10X HRP Label||1 x 1ml|
|20X Buffer||1 x 20ml|
|Extraction Buffer||1 x 15ml|
|FH Microplate||1 unit|
|Hela Lysate Control (200ug)||1 x 200µg|
|TMB Development Solution||1 x 12ml|
Our Abpromise guarantee covers the use of ab157399 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|Sandwich ELISA||Use at an assay dependent concentration.|
ab157399 has not yet been referenced specifically in any publications.
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"