FH Polyclonal Antibody, APC-Cy7 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

Clonality

Polyclonal

Isotype

IgG

Conjugation

APC-Cy7

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

650nm/780nm

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

Curated Selection

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