ALS2 Antikörper (AA 1384-1440) (AbBy Fluor® 350)

Produktnummer ABIN1392873

Produktdetails anti-ALS2 Antikörper (AbBy Fluor® 350)

Target: ALS2 (Amyotrophic Lateral Sclerosis 2 (Juvenile) (ALS2))

Bindungsspezifität: AA 1384-1440

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser ALS2 Antikörper ist konjugiert mit AbBy Fluor® 350

Applikation: Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Homologie: Human,Mouse,Rat,Dog,Cow,Sheep,Pig,Horse,Rabbit

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human ALS2

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Konservierungsmittel: ProClin

Vorsichtsmaßnahmen: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Haltbarkeit: 12 months

Details zu ALS2

Target: ALS2 (Amyotrophic Lateral Sclerosis 2 (Juvenile) (ALS2))

Andere Bezeichnung: ALS2/Alsin (ALS2 Produkte)

Hintergrund:

Synonyms: ALS 2, ALS2, ALS2_HUMAN, ALS2CR6, Alsin, ALSJ, Amyotrophic lateral sclerosis 2 juvenile, Amyotrophic lateral sclerosis 2 juvenile chromosome region candidate 6, Amyotrophic lateral sclerosis 2 chromosomal region candidate gene 6 protein, Amyotrophic lateral sclerosis 2 protein, Amyotrophic lateral sclerosis protein 2, FLJ31851, IAHSP, KIAA1563, MGC87187, PLSJ.

Background: Mutations in the ALS2 gene result in a number of juvenile recessive motor neuron diseases (MNDs), including juvenile primary lateral sclerosis (JPLS), a recessive form of amyotrophic lateral sclerosis (ALS2), infantile onset ascending hereditary spastic paralysis (IAHSP), and a form of complicated hereditary spastic paraplegia (cHSP). The ALS2 gene encodes the Alsin protein. Alsin acts as a guanine nucleotide exchange factor for Rab5, a modulator of the endocytic pathway. Alsin is a cytosolic protein that is associated with small, punctate membrane structures. Therefore, Alsin may mediate membrane transport events, potentially linking endocytic processes and actin cytoskeleton remodeling. The ALS2 C-terminal-like protein (ALS2CL) also modulates Rab 5 activity.

Pathways: Skeletal Muscle Fiber Development