{"product_id":"pn-7100a","title":"Rabbit anti-Human Amyotrophic lateral sclerosis protein 2 (ALS2) IgG fraction polyclonal antibody","description":"\u003cp\u003eIgG fraction from rabbit polyclonal antibody obtained by immunizing rabbits with a synthetic peptide (LKACYYQIQREKLN) corresponding to amino acids 1644-1657 of human ALS2. The IgG fraction was purified using Protein G-Sepharose.\u003c\/p\u003e\u003cul\u003e\n\u003cli\u003e\n\u003cstrong\u003eStorage:\u003c\/strong\u003e Store at -20°C.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eStability:\u003c\/strong\u003e Stable at least one year at -20°C. Avoid repeated freezing and thawing.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eBiological activity:\u003c\/strong\u003e This polyclonal antibody reacts in ELISA (1: 50 dilution) using the immunizing peptide. Amyotrophic lateral sclerosis protein 2 (ALS2) or Alsin is a 184 kDa protein that contains three guanine-nucleotide exchange factor domains and may act as a GTPase regulator. ALS2 dysfunction affects endosome trafficking through a Rab5 small GTPases family-mediated mechanism. It is a causative gene for a juvenile autosomal recessive form of motor neuron diseases, including amyotrophic lateral sclerosis 2. This disorder is characterized by a progressive degeneration of the upper motor neurons of the motor cortex and the lower motor neurons of the brain stem and spinal cord.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eFormulation:\u003c\/strong\u003e Solution at 1 mg\/mL in PBS. Some of the liquid in the vial could have evaporated with changes in the final volume. However, the mass of the protein is still inside the vial.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eReferences:\u003c\/strong\u003e Yang, Y. et al. (2001) Nat. Genet. 29, 160-165. Hadano, S. et al. (20001) Nat. Genet. 166-173. Chandran, J. et al. (2007) Mol. Neurobiol. 224-231. Hadano, S. et al. (2007) Neurochem. Int. 51, 74-84.\u003c\/li\u003e\n\u003c\/ul\u003e\u003cp class=\"ab-datasheet-link\"\u003e\u003ca href=\"https:\/\/helix-chartreuse-ypja.squarespace.com\/s\/Austral_Biologicals_PN-7100A_Datasheet.pdf\" target=\"_blank\" rel=\"noopener\"\u003e\u003cstrong\u003eDownload technical datasheet (PDF)\u003c\/strong\u003e\u003c\/a\u003e\u003c\/p\u003e","brand":"Austral Biologicals","offers":[{"title":"1mg","offer_id":52517555634478,"sku":"PN-7100a-9","price":425.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/1006\/2846\/3918\/files\/austral-biologicals-pn-7100a-1mg.png?v=1788008140","url":"https:\/\/www.australbiologicals.com\/products\/pn-7100a","provider":"Austral Biologicals","version":"1.0","type":"link"}