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anti-ALS2 antibody :: Goat Alsin/ALS2 Polyclonal Antibody

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Catalog # MBS420754
Unit / Price
  0.1 mg  /  $300 +1 FREE 8GB USB
Immunohistochemistry (IHC)
Product Name

Alsin/ALS2, Polyclonal Antibody

Also Known As

Goat anti-Alsin/ALS2 Antibody

Product Synonym Names
ALS2; amyotrophic lateral sclerosis 2 (juvenile); ALSJ; PLSJ; IAHSP; KIAA1563; ALS2CR6; FLJ31851; MGC87187; alsin; ALS2 antibody; amyotrophic lateral sclerosis 2 (juvenile) antibody; ALSJ antibody; PLSJ antibody; IAHSP antibody; KIAA1563 antibody; ALS2CR6 antibody; FLJ31851 antibody; MGC87187 antibody; alsin antibody; Alsin; Alsin / ALS2
Antibody/Peptide Pairs
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Immunogen Sequence Length
1657
Immunogen Sequence
LKACYYQIQR EKLN
OMIM
205100
Clonality
Polyclonal
Host
Goat
Species Reactivity
Tested: Human; Expected from sequence similarity: Human, Mouse, Rat, Dog, Cow
Purity/Purification
Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Form/Format
Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.
Concentration
100ug specific antibody in 200ul (lot specific)
Immunogen
Peptide with sequence LKACYYQIQREKLN, from the C Terminus of the protein sequence according to NP_065970.2.
Epitope
C Terminus
Preparation and Storage
Aliquot and store at -20 degree C. Minimize freezing and thawing.
ISO Certification
Manufactured in an ISO 9001:2015 Certified Laboratory.
Other Notes
Small volumes of anti-ALS2 antibody 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.
Applications Tested/Suitable for anti-ALS2 antibody
Peptide ELISA (EIA), Immunohistochemistry (IHC)
Application Notes for anti-ALS2 antibody
Peptide ELISA: Antibody detection limit dilution 1: 8000.
Immunohistochemistry: In paraffin embedded Human Cortex shows pixulate cytoplasm staining in some neuronal cells. Recommended concentration, 3-5ug/ml.
Western Blot: No signal obtained yet but low background observed in Human Brain extracts at upto 1ug/ml.

Immunohistochemistry (IHC) of anti-ALS2 antibody
(3.8ug/ml) staining of paraffin embedded Human Cortex. Steamed antigen retrieval with citrate buffer pH 6, AP-staining.
anti-ALS2 antibody Immunohistochemistry (IHC) (IHC) image
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; GEFs, Rab

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
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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.

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