Amyotrophic Lateral Sclerosis 2 (Juvenile) (ALS2) (C-Term) antibody

Details for Product No. ABIN374168
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Antigen
Synonyms als2, si:dkey-33m14.1, ALS2CR6, ALSJ, IAHSP, PLSJ, 3222402C23Rik, 9430073A21Rik, Als2cr6, Alsin, mKIAA1563
Epitope
C-Term
(6), (3), (2), (2), (2), (1), (1), (1), (1), (1)
Reactivity
Cow (Bovine), Dog (Canine), Human, Mouse (Murine), Rat (Rattus)
(37), (20), (18), (14), (13), (12)
Host
Goat
(30), (6), (6)
Clonality
Polyclonal
Conjugate
Un-conjugated
(1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Application
Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))
(24), (14), (10), (7), (6), (3)
Pubmed 1 reference available
Quantity 0.1 mg
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Catalog No. ABIN374168
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Immunogen Peptide from C Terminus of the protein sequence according to NP_065970.2 Genename: ALS2
Sequence LKACYYQIQR EKLN
Specificity This antibody detects Alsin / ALS2 at C-term.
Purification Ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide
Alternative Name Alsin / ALS2
Background ALS2 specifically binds to, and functions as a guanine nucleotide exchange factor (GEF) for the small GTPase RAB5. ALS2 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 localizes with RAB5 on early endosomal compartments, and functions as a modulator for endosomal dynamics. Loss of ALS2 function might underlie neuronal dysfunction and degeneration in a number of motor neuron diseases.
Alternate names: ALS2CR6, Amyotrophic lateral sclerosis 2 chromosomal region candidate gene 6 protein, Amyotrophic lateral sclerosis protein 2, KIAA1563
Gene ID 57679
NCBI Accession NP_065970
UniProt Q96Q42
Application Notes Peptide ELISA: Limit dilution 1: 8000. Immunohistochemistry: 3-5 µg/ml. In paraffin embedded Human Cortex shows pixulatecytoplasm staining in some neuronal cells. Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user.
Restrictions For Research Use only
Format Liquid
Concentration 0.5 mg/mL
Buffer Tris buffered saline, 0.02% sodium azide, pH7.3 with 0.5% bovine serum albumin
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Handling Advice Avoid repeated freezing and thawing.
Storage 4 °C/-20 °C
Storage Comment Store at 2 - 8 °C for up to one month or (in aliquots) at -20 °C.
Expiry Date 12 months
Supplier Images
anti-Amyotrophic Lateral Sclerosis 2 (Juvenile) (ALS2) (C-Term) antibody anti-Amyotrophic Lateral Sclerosis 2 (Juvenile) (ALS2) (C-Term) antibody
Background publications Yang, Hentati, Deng et al.: "The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis." in: Nature genetics, Vol. 29, Issue 2, pp. 160-5, 2001 (PubMed).

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