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

Scan QR to view Datasheet Catalog #    MBS420754
Immunohistochemistry (IHC)
Unit / Price
0.1 mg  /  $300 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 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
 Product Gene Name   

anti-ALS2 antibody

[Similar Products]
 Antibody/Peptide Pairs    Alsin / ALS2 peptide (MBS426900) is used for blocking the activity of Alsin/ALS2 antibody (MBS420754)
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Immunogen Sequence    LKACYYQIQR EKLN
 OMIM    205100
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 Clonality    Polyclonal
 Host    Goat
 Species Reactivity    Tested: Human; Expected from sequence similarity: Human, Mouse, Rat, Dog, Cow
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 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)
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 Immunogen    Peptide with sequence LKACYYQIQREKLN, from the C Terminus of the protein sequence according to NP_065970.2.
 Epitope    C Terminus
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 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.
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 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.
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 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
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NCBI/Uniprot data below describe general gene information for ALS2. It may not necessarily be applicable to this product.
 NCBI GI #    40316935
 NCBI GeneID    57679
 NCBI Accession #    NP_065970.2 [Other Products]
 NCBI GenBank Nucleotide #    NM_020919.3 [Other Products]
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 UniProt Secondary Accession #    Q53TT1; Q53TV2; Q8N1E0; Q96PC4; Q96Q41; Q9H973; Q9HCK9 [Other Products]
 UniProt Related Accession #    Q96Q42 [Other Products]
 Molecular Weight    86,782 Da
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 NCBI Official Full Name    alsin isoform 1
 NCBI Official Synonym Full Names    ALS2, alsin Rho guanine nucleotide exchange factor
 NCBI Official Symbol    ALS2 [Similar Products]
 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    Alsin
 UniProt Gene Name    ALS2 [Similar Products]
 UniProt Synonym Gene Names    ALS2CR6; KIAA1563 [Similar Products]
 UniProt Entry Name    ALS2_HUMAN
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 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]
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 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
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 Research Articles on ALS2    1. We identified a novel homozygous splice-site mutation (c.3512+1G>A) in the ALS2 gene (NM_020919.3) encoding alsin that segregated with the disease in this family
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 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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Products associated with anti-ALS2 antibodyPathways associated with anti-ALS2 antibody
 Reference Product  PubMed Publications
 SOD1 antibody  >36 publications with ALS2 and SOD1
 SETX antibody  >12 publications with ALS2 and SETX
 RAC1 antibody  >9 publications with ALS2 and RAC1
 RABGEF1 antibody  >2 publications with ALS2 and RABGEF1
 EEA1 antibody  >2 publications with ALS2 and EEA1
 ALS2CR8 antibody  >1 publications with ALS2 and ALS2CR8
 GRIP1 antibody  >1 publications with ALS2 and GRIP1
 RAB5A antibody  >1 publications with ALS2 and RAB5A
 Products by Pathway  Pathway Diagram
 Amyotrophic Lateral Sclerosis (ALS) Pathway antibodies  Amyotrophic Lateral Sclerosis (ALS) Pathway Diagram
 Amyotrophic Lateral Sclerosis (ALS) Pathway antibodies  Amyotrophic Lateral Sclerosis (ALS) Pathway Diagram
 Amyotrophic Lateral Sclerosis (ALS) Pathway antibodies  Amyotrophic Lateral Sclerosis (ALS) Pathway Diagram
Diseases associated with anti-ALS2 antibodyOrgans/Tissues associated with anti-ALS2 antibody
 Disease Name  Pubmed Publications
 Nervous System Diseases Antibodies  >91 publications with ALS2 and Nervous System Diseases
 Primary lateral sclerosis juvenile Antibodies  >55 publications with ALS2 and Primary lateral sclerosis juvenile
 Disease Models, Animal Antibodies  >17 publications with ALS2 and Disease Models, Animal
 Atrophy Antibodies  >9 publications with ALS2 and Atrophy
 Nerve Degeneration Antibodies  >7 publications with ALS2 and Nerve Degeneration
 Movement Disorders Antibodies  >6 publications with ALS2 and Movement Disorders
 Weight Loss Antibodies  >4 publications with ALS2 and Weight Loss
 Inflammation Antibodies  >4 publications with ALS2 and Inflammation
 Hereditary spastic paralysis, infantile onset ascending Antibodies  >4 publications with ALS2 and Hereditary spastic paralysis, infantile onset ascending
 Amyotrophic Lateral Sclerosis 2, Juvenile Antibodies  >3 publications with ALS2 and Amyotrophic Lateral Sclerosis 2, Juvenile
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >27 publications with ALS2 and Brain
 Muscle Antibodies  >13 publications with ALS2 and Muscle
 Bone Antibodies  >3 publications with ALS2 and Bone
 Mouth Antibodies  >3 publications with ALS2 and Mouth
 Lung Antibodies  >3 publications with ALS2 and Lung
 Heart Antibodies  >3 publications with ALS2 and Heart
 Blood Antibodies  >2 publications with ALS2 and Blood
 Eye Antibodies  >2 publications with ALS2 and Eye
 Vascular Antibodies  >2 publications with ALS2 and Vascular
 Skin Antibodies  >1 publications with ALS2 and Skin
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