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ALS2 Polyclonal Antibody, APC Conjugated

Product Specifications

Background

Mutations in the ALS2 gene result in a number of juvenile recessive motor neuron diseases (MNDs), including juvenile primary lateral sclerosis (JPLS), a recessive form of amyotrophic lateral sclerosis (ALS2) ; infantile onset ascending hereditary spastic paralysis (IAHSP) ; and a form of complicated hereditary spastic paraplegia (cHSP) . The ALS2 gene encodes the Alsin protein. Alsin acts as a guanine nucleotide exchange factor for Rab5, a modulator of the endocytic pathway. Alsin is a cytosolic protein that is associated with small, punctate membrane structures. Therefore, Alsin may mediate membrane transport events, potentially linking endocytic processes and actin cytoskeleton remodeling. The ALS2 C-terminal-like protein (ALS2CL) also modulates Rab 5 activity.

Synonyms

ALS 2; ALS2; ALS2_HUMAN; ALS2CR6; Alsin; ALSJ; Amyotrophic lateral sclerosis 2 juvenile; Amyotrophic lateral sclerosis 2 juvenile chromosome region candidate 6; Amyotrophic lateral sclerosis 2 chromosomal region candidate gene 6 protein; Amyotrophic lateral sclerosis 2 protein; Amyotrophic lateral sclerosis protein 2; FLJ31851; IAHSP; KIAA1563; MGC87187; PLSJ.

Cellular Locus

Cytoplasm

Host

Rabbit

Immunogen

1384-1440/1657

Target

ALS2

Clonality

Polyclonal

Isotype

IgG

Conjugation

APC

Source

KLH conjugated synthetic peptide derived from human ALS2

Applications

IF (IHC-P), IF (IHC-F), IF (ICC)

Purification

Purified by Protein A.

Excitation Emission

650nm/660nm

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.

Predicted Cross Reactivity

Human, Mouse, Rat, Dog, Cow, Sheep, Pig, Horse, Rabbit

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