ArticleScientific reports2017
L-leucine and SPNS1 coordinately ameliorate dysfunction of autophagy in mouse and human Niemann-Pick type C disease.
Article in Scientific reports, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 32 citations in OpenAlex.
- Inflammatory cytokines mediate the common genetic and immunological background between rheumatoid arthritis and osteoporosis.Clinical rheumatology · 2026Article
- Rag GTPases control lysosomal acidification by regulating v-ATPase assembly in Drosophila.The Journal of biological chemistry · 2025Article
- Spns1-dependent endocardial lysosomal function drives valve morphogenesis through Notch1-signaling.iScience · 2024Article
- Spns1 is an iron transporter essential for megalin-dependent endocytosis.American journal of physiology. Renal physiology · 2024Article
- Lack of SPNS1 results in accumulation of lysolipids and lysosomal storage disease in mouse models.JCI insight · 2024Article
- Autophagic lysosome reformation in health and disease.Autophagy · 2023Review
- Autophagy-linked plasma and lysosomal membrane protein PLAC8 is a key host factor for SARS-CoV-2 entry into human cells.The EMBO journal · 2022Article
- Bioinformatics-based identification of SPNS3 (Spinster homolog 3) as a prognostic biomarker of apoptosis resistance in acute myeloid leukemia.Bioengineered · 2021Article
- Lysosomal Function Impacts the Skeletal Muscle Extracellular Matrix.Journal of developmental biology · 2021Article
- Review
- Prognostic Significance of Cytoplasmic SPNS2 Expression in Patients with Oral Squamous Cell Carcinoma.Medicina (Kaunas, Lithuania) · 2021Article
- In-Silico Study of Immune System Associated Genes in Case of Type-2 Diabetes With Insulin Action and Resistance, and/or Obesity.Frontiers in endocrinology · 2021Article
- Acetyl-leucine slows disease progression in lysosomal storage disorders.Brain communications · 2021Article
- Identification of an autophagy-related gene signature that can improve prognosis of hepatocellular carcinoma patients.BMC cancer · 2020Article
- Beneficial Effects of Acetyl-DL-Leucine (ADLL) in a Mouse Model of Sandhoff Disease.Journal of clinical medicine · 2020Article
- Prognostic significance of Spinster homolog gene family in acute myeloid leukemia.Journal of Cancer · 2020Article
- Glucose Transport and Transporters in the Endomembranes.International journal of molecular sciences · 2019Review
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11 authors at 6 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Lysosomal storage disorders are characterized by progressive accumulation of undigested macromolecules within the cell due to lysosomal dysfunction. 573C10 is a Schwann cell line derived from a mouse model of Niemann-Pick type C disease-1, NPC (-/-). Under serum-starved conditions, NPC (-/-) cells manifested impaired autophagy accompanied by an increase in the amount of p62 and lysosome enlargement. Addition of L-leucine to serum-starved NPC (-/-) cells ameliorated the enlargement of lysosomes and the p62 accumulation. Similar autophagy defects were observed in NPC (-/-) cells even without serum starvation upon the knockdown of Spinster-like 1 (SPNS1), a putative transporter protein thought to function in lysosomal recycling. Conversely, SPNS1 overexpression impeded the enlargement of lysosomes, p62 accumulation and mislocalization of the phosphorylated form of the mechanistic Target of rapamycin in NPC (-/-) cells. In addition, we found a reduction in endogenous SPNS1 expression in fibroblasts derived from NPC-1 patients compared with normal fibroblasts. We propose that SPNS1-dependent L-leucine export across the lysosomal membrane is a key step for triggering autophagy, and that this mechanism is impaired in NPC-1.
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