FH Polyclonal Antibody, RBITC Conjugated
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
RBITC
Source
KLH conjugated synthetic peptide derived from human Fumarate hydratase (447-496aa)
Applications
IF (IHC-P), IF (IHC-F), IF (ICC)
Purification
Purified by Protein A.
Excitation Emission
570nm/595nm
Concentration
1µg/µl
Dilution
IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)
Buffer
Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Modification
Unmodified
Storage Conditions
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Gene ID URL
2271
Predicted Cross Reactivity
Human, Dog, Cow, Sheep, Pig, Horse, Rabbit
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