This antibody is purified through a protein A column, followed by peptide affinity purification.
Immunogène
This CTNS antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 341-367 amino acids from the C-terminal region of human CTNS.
CTNS
Reactivité: Humain
WB
Hôte: Lapin
Polyclonal
unconjugated
Indications d'application
WB: 1:1000
Restrictions
For Research Use only
Format
Liquid
Buffer
Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.
Agent conservateur
Sodium azide
Précaution d'utilisation
This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Stock
4 °C,-20 °C
Date de péremption
6 months
Taranta, Wilmer, van den Heuvel, Bencivenga, Bellomo, Levtchenko, Emma: "Analysis of CTNS gene transcripts in nephropathic cystinosis." dans: Pediatric nephrology (Berlin, Germany), Vol. 25, Issue 7, pp. 1263-7, (2010) (PubMed).
Kumar, Bachhawat: "A futile cycle, formed between two ATP-dependant gamma-glutamyl cycle enzymes, gamma-glutamyl cysteine synthetase and 5-oxoprolinase: the cause of cellular ATP depletion in nephrotic cystinosis?" dans: Journal of biosciences, Vol. 35, Issue 1, pp. 21-5, (2010) (PubMed).
Bellomo, Corallini, Pastore, Palma, Laurenzi, Emma, Taranta: "Modulation of CTNS gene expression by intracellular thiols." dans: Free radical biology & medicine, Vol. 48, Issue 7, pp. 865-72, (2010) (PubMed).
Aldahmesh, Humeidan, Almojalli, Khan, Rajab, AL-Abbad, Meyer, Alkuraya: "Characterization of CTNS mutations in Arab patients with cystinosis." dans: Ophthalmic genetics, Vol. 30, Issue 4, pp. 185-9, (2009) (PubMed).
Macías-Vidal, Rodés, Hernández-Pérez, Vilaseca, Coll: "Analysis of the CTNS gene in 32 cystinosis patients from Spain." dans: Clinical genetics, Vol. 76, Issue 5, pp. 486-9, (2009) (PubMed).
This gene encodes a seven-transmembrane domain protein that functions to transport cystine out of lysosomes. Its activity is driven by the H+ electrochemical gradient of the lysosomal membrane. Mutations in this gene cause cystinosis, a lysosomal storage disorder. Alternative splicing results in multiple transcript variants.