FH Polyclonal Antibody
Product Specifications
Background
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.
Synonyms
Mitochondrial; FH; Fumarase; Fumarate hydratase; Fumarate hydratase mitochondrial; FUMH_HUMAN; HLRCC; LRCC; MCL; MCUL 1; MCUL1; Multiple hereditary cutaneous leiomyomata.
Gene ID
2271
Cellular Locus
Cytoplasm
Host
Rabbit
Cross Reactivity
Mouse, Rat
Immunogen
411-510/510
Target
FH
Clonality
Polyclonal
Isotype
IgG
Conjugation
Unconjugated
Source
KLH conjugated synthetic peptide derived from human Fumarate hydratase (447-496aa)
Applications
ELISA, IHC-P, IHC-F, IF (IHC-P), IF (IHC-F), IF (ICC)
Purification
Purified by Protein A.
Concentration
1µg/µl
Dilution
ELISA (1:500-1000), IHC-P (1:200-400), IHC-F (1:100-500), IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)
Buffer
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Modification
Unmodified
Storage Conditions
Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
Gene ID URL
2271
Predicted Cross Reactivity
Human, Dog, Cow, Sheep, Pig, Horse, Rabbit
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