Anti-ALS2 antibody (AA38930)

$187.00


Alternative names Alsin, Amyotrophic lateral sclerosis 2 chromosomal region candidate gene 6 protein, Amyotrophic lateral sclerosis 2 protein, ALS2, ALS2CR6, KIAA1563, ALS2_HUMAN, Q96Q42, 57679
Source 1657
Species Reactivity Human
Applications IF/ICC, IHC, WB
Host Rabbit
Class Polyclonal
Conjugated Unconjugated


Description

Product name Anti-ALS2 antibody (AA38930)
Catalog number AA38930
Brand family Ango
Product type Primary Antibody
Antibody Alternative Names Alsin antibody, Amyotrophic lateral sclerosis 2 chromosomal region candidate gene 6 protein antibody, Amyotrophic lateral sclerosis 2 protein antibody, ALS2 antibody, ALS2CR6 antibody, KIAA1563 antibody, ALS2_HUMAN antibody


Biological Information

Immunogen Recombinant protein of human ALS2
Concentration 1 mg/ml
Isotype IgG
Application Notes WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:100
Purification method Purified by affinity chromatography


Target

Target Name ALS2
SwissProt ID Link Q96Q42
Function May act as a GTPase regulator. Controls survival and growth of spinal motoneurons.
Involvement in Disease Amyotrophic lateral sclerosis 2: A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases. The disease is caused by mutations affecting the gene represented in this entry. Juvenile primary lateral sclerosis: A neurodegenerative disorder which is closely related to but clinically distinct from amyotrophic lateral sclerosis. It is a progressive paralytic disorder which results from dysfunction of the upper motor neurons while the lower neurons are unaffected. The disease is caused by mutations affecting the gene represented in this entry. Infantile-onset ascending spastic paralysis: Characterized by progressive spasticity and weakness of limbs. The disease is caused by mutations affecting the gene represented in this entry.
Subunit Structure Forms a heteromeric complex with ALS2CL. Interacts with ALS2CL.
Sequence Similarities Contains 1 DH (DBL-homology) domain. Contains 8 MORN repeats. Contains 1 PH domain. Contains 5 RCC1 repeats. Contains 1 VPS9 domain.


Poduct Presentation

Form Liquid
Preservative 0.02% (w/v) sodium azide
Storage buffer PBS with 50% glycerol, pH 7.3


Storage and Shipping Information

Storage Store at -20 or -80 C. Avoid multiple freeze/thaw cycles.