Cystinosin, the protein defective in cystinosis, is a H(+)-driven lysosomal cystine transporter
about
LAAT-1 is the lysosomal lysine/arginine transporter that maintains amino acid homeostasisDecreased intracellular ATP content and intact mitochondrial energy generating capacity in human cystinotic fibroblastsThe proteome of lysosomesMolecular characterisation of 'transmembrane protein 192' (TMEM192), a novel protein of the lysosomal membraneIdentification and subcellular localization of a new cystinosin isoformAmino acids in the cultivation of mammalian cellsRole of amino acid transporters in amino acid sensingLysosomal membrane proteins and their central role in physiologyIs genetic rescue of cystinosis an achievable treatment goal?Nephropathic cystinosis: an international consensus documentBrief reports: Lysosomal cross-correction by hematopoietic stem cell-derived macrophages via tunneling nanotubesPhagocytic receptor CED-1 initiates a signaling pathway for degrading engulfed apoptotic cellsUNC-16 (JIP3) Acts Through Synapse-Assembly Proteins to Inhibit the Active Transport of Cell Soma Organelles to Caenorhabditis elegans Motor Neuron Axons.Carboxyl-Terminal SSLKG Motif of the Human Cystinosin-LKG Plays an Important Role in Plasma Membrane SortingDisruption of a cystine transporter downregulates expression of genes involved in sulfur regulation and cellular respiration.Heptahelical protein PQLC2 is a lysosomal cationic amino acid exporter underlying the action of cysteamine in cystinosis therapyQuantitative in vivo and ex vivo confocal microscopy analysis of corneal cystine crystals in the Ctns knockout mouseEndo-lysosomal dysfunction in human proximal tubular epithelial cells deficient for lysosomal cystine transporter cystinosinProbes of the catalytic site of cysteine dioxygenaseExpression and lysosomal targeting of CLN7, a major facilitator superfamily transporter associated with variant late-infantile neuronal ceroid lipofuscinosisIntralysosomal cystine accumulation in mice lacking cystinosin, the protein defective in cystinosisSulfide Oxidation by O2: Synthesis, Structure and Reactivity of Novel Sulfide-Incorporated Fe(II) Bis(imino)pyridine ComplexesRhoA GTPase regulates M-cadherin activity and myoblast fusionAn organelle gatekeeper function for Caenorhabditis elegans UNC-16 (JIP3) at the axon initial segment.Upregulation of the Rab27a-dependent trafficking and secretory mechanisms improves lysosomal transport, alleviates endoplasmic reticulum stress, and reduces lysosome overload in cystinosis.Time course of pathogenic and adaptation mechanisms in cystinotic mouse kidneysLysosomal function in macromolecular homeostasis and bioenergetics in Parkinson's disease.Analysis of CTNS gene transcripts in nephropathic cystinosisCystinosin, MPDU1, SWEETs and KDELR belong to a well-defined protein family with putative function of cargo receptors involved in vesicle traffickingDisrupted in renal carcinoma 2 (DIRC2), a novel transporter of the lysosomal membrane, is proteolytically processed by cathepsin L.Diversity of human hair pigmentation as studied by chemical analysis of eumelanin and pheomelanin.Corneal transduction by intra-stromal injection of AAV vectors in vivo in the mouse and ex vivo in human explants.A chimera carrying the functional domain of the orphan protein SLC7A14 in the backbone of SLC7A2 mediates trans-stimulated arginine transport.Maternal uniparental heterodisomy of chromosome 17 in a patient with nephropathic cystinosis.Stem cell microvesicles transfer cystinosin to human cystinotic cells and reduce cystine accumulation in vitro.Inhibition of intracellular clusterin attenuates cell death in nephropathic cystinosis.Treatment of corneal cystine crystal accumulation in patients with cystinosis.Carnitine Profile and Effect of Suppletion in Children with Renal Fanconi Syndrome due to Cystinosis.Lysosomal physiology.Impairment of chaperone-mediated autophagy leads to selective lysosomal degradation defects in the lysosomal storage disease cystinosis
P2860
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P2860
Cystinosin, the protein defective in cystinosis, is a H(+)-driven lysosomal cystine transporter
description
2001 nî lūn-bûn
@nan
2001 թուականի Նոյեմբերին հրատարակուած գիտական յօդուած
@hyw
2001 թվականի նոյեմբերին հրատարակված գիտական հոդված
@hy
2001年の論文
@ja
2001年論文
@yue
2001年論文
@zh-hant
2001年論文
@zh-hk
2001年論文
@zh-mo
2001年論文
@zh-tw
2001年论文
@wuu
name
Cystinosin, the protein defect ...... lysosomal cystine transporter
@ast
Cystinosin, the protein defect ...... lysosomal cystine transporter
@en
Cystinosin, the protein defect ...... lysosomal cystine transporter.
@nl
type
label
Cystinosin, the protein defect ...... lysosomal cystine transporter
@ast
Cystinosin, the protein defect ...... lysosomal cystine transporter
@en
Cystinosin, the protein defect ...... lysosomal cystine transporter.
@nl
prefLabel
Cystinosin, the protein defect ...... lysosomal cystine transporter
@ast
Cystinosin, the protein defect ...... lysosomal cystine transporter
@en
Cystinosin, the protein defect ...... lysosomal cystine transporter.
@nl
P2860
P50
P3181
P356
P1433
P1476
Cystinosin, the protein defect ...... lysosomal cystine transporter
@en
P2093
P2860
P304
P3181
P356
10.1093/EMBOJ/20.21.5940
P407
P577
2001-11-01T00:00:00Z