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ALS2 peptide :: Alsin / ALS2 Peptide

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Catalog # MBS426900
Unit / Price
  0.1 mg  /  $190 +1 FREE 8GB USB
ALS2 peptide
Product Name

Alsin / ALS2, Peptide

Also Known As

Alsin / ALS2 Immunizing Peptide

Product Synonym Names
ALS2; amyotrophic lateral sclerosis 2 (juvenile); ALSJ; PLSJ; IAHSP; KIAA1563; ALS2CR6; FLJ31851; MGC87187; alsin; Alsin; Alsin / ALS2
Product Gene Name
Antibody/Peptide Pairs
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence Length
1657
Sequence
LKACYYQIQR EKLN
OMIM
205100
Species Reactivity
Human, Mouse, Rat, Dog, Cow
Form/Format
100ug of dried peptide
Preparation and Storage
Shipped at ambient temperature, store at -20 degree C
ISO Certification
Manufactured in an ISO 9001:2015 Certified Laboratory.
Other Notes
Small volumes of ALS2 peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
NCBI/Uniprot data below describe general gene information for ALS2. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
86,782 Da
NCBI Official Full Name
alsin isoform 1
NCBI Official Synonym Full Names
ALS2, alsin Rho guanine nucleotide exchange factor
NCBI Official Symbol
NCBI Official Synonym Symbols
ALSJ; PLSJ; IAHSP; ALS2CR6
  [Similar Products]
NCBI Protein Information
alsin
UniProt Protein Name
Alsin
UniProt Synonym Protein Names
Amyotrophic lateral sclerosis 2 chromosomal region candidate gene 6 protein; Amyotrophic lateral sclerosis 2 protein
Protein Family
UniProt Gene Name
UniProt Synonym Gene Names
ALS2CR6; KIAA1563  [Similar Products]
UniProt Entry Name
ALS2_HUMAN
NCBI Summary for ALS2
The protein encoded by this gene contains an ATS1/RCC1-like domain, a RhoGEF domain, and a vacuolar protein sorting 9 (VPS9) domain, all of which are guanine-nucleotide exchange factors that activate members of the Ras superfamily of GTPases. The protein functions as a guanine nucleotide exchange factor for the small GTPase RAB5. The protein localizes with RAB5 on early endosomal compartments, and functions as a modulator for endosomal dynamics. Mutations in this gene result in several forms of juvenile lateral sclerosis and infantile-onset ascending spastic paralysis. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2008]
UniProt Comments for ALS2
ALS2: May act as a GTPase regulator. Controls survival and growth of spinal motoneurons. Defects in ALS2 are the cause of amyotrophic lateral sclerosis type 2 (ALS2). ALS2 is a familial form of amyotrophic lateral sclerosis, a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. 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 cases leading to familial forms. Defects in ALS2 are the cause of juvenile primary lateral sclerosis (JPLS). JPLS is 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 of the motor cortex while the lower neurons are unaffected. Defects in ALS2 are the cause of infantile-onset ascending spastic paralysis (IAHSP). IAHSP is characterized by progressive spasticity and weakness of limbs. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: GEFs, Rab; GEFs

Chromosomal Location of Human Ortholog: 2q33.1

Cellular Component: axon; cell soma; centrosome; cytosol; dendrite; dendritic spine; early endosome; growth cone; lamellipodium; membrane; postsynaptic density; protein complex; ruffle; vesicle

Molecular Function: guanyl-nucleotide exchange factor activity; protein binding; protein homodimerization activity; protein serine/threonine kinase activator activity; Rab GTPase binding; Rab guanyl-nucleotide exchange factor activity; Rac guanyl-nucleotide exchange factor activity; Ran guanyl-nucleotide exchange factor activity

Biological Process: axonogenesis; behavioral fear response; cell death; endosome organization and biogenesis; endosome transport; in utero embryonic development; locomotory behavior; neurite morphogenesis; neuromuscular junction development; positive regulation of GTPase activity; positive regulation of protein kinase activity; positive regulation of Rac protein signal transduction; protein localization; Rac protein signal transduction; receptor recycling; regulation of endosome size; regulation of Rho protein signal transduction; response to oxidative stress; synaptic transmission, glutamatergic; vesicle organization and biogenesis

Disease: Amyotrophic Lateral Sclerosis 2, Juvenile; Primary Lateral Sclerosis, Juvenile; Spastic Paralysis, Infantile-onset Ascending
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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