This antibody reacts specifically with Fumarate hydratase/FH
Cross reactive species
Human (Homo sapiens), Mouse (Mus musculus), Rat (Rattus norvegicus)
Antigen Source
KLH conjugated synthetic peptide derived from human Fumarate hydratase (447-496aa)
Storage
Water buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.
Description
This antibody needs to be stored at + 4°C in a fridge short term in a concentrated dilution. Freeze thaw will destroy a percentage in every cycle and should be avoided.
Defects in FH are the cause of fumarase deficiency (FHD)also known as fumaricaciduria. FHD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia.
Cross Reactive Species details
No significant cross reactivity has been observed for this antibody for the tested species. However, note that due to limited knowledge it is impossible to predict with 100% guarantee that the antibody does not corss react with any other species.
Advisory
Avoid freeze/thaw cycles as they may denaturate the polypeptide chains of the antibody, thus reducing its reactivity, specificity and sensitivity. For antibodies that are in liquid form or reconstituted lyophilized antibodies small amounts could become entrapped on the seal or the walls of the tube. Prior to use briefly centrifuge the vial to gather all the solution on the bottom.
