胱氨酸病白细胞中富含溶酶体的分离组分的胱氨酸外排缺陷。 - Suppr

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超能文献

Defective cystine exodus from isolated lysosome-rich fractions of cystinotic leucocytes.

作者信息

Gahl W A, Tietze F, Bashan N, Steinherz R, Schulman J D

出版信息

J Biol Chem. 1982 Aug 25;257(16):9570-5.

PMID:7107582

Abstract

摘要

相似文献

Defective cystine exodus from isolated lysosome-rich fractions of cystinotic leucocytes.胱氨酸病白细胞中富含溶酶体的分离组分的胱氨酸外排缺陷。

J Biol Chem. 1982 Aug 25;257(16):9570-5.

Effects of cysteamine and other thiols on cystine egress from isolated lysosome-rich fractions of cystinotic and normal leucocytes.半胱胺及其他硫醇对胱氨酸从胱氨酸病患者和正常白细胞富含溶酶体的分离组分中流出的影响。

Clin Chim Acta. 1983 Jul 31;132(1):17-22. doi: 10.1016/0009-8981(83)90228-0.

Cystine accumulation and clearance by normal and cystinotic leukocytes exposed to cystine dimethyl ester.正常白细胞和胱氨酸病白细胞对胱氨酸二甲酯的胱氨酸积累及清除情况

Proc Natl Acad Sci U S A. 1982 Jul;79(14):4446-50. doi: 10.1073/pnas.79.14.4446.

The effect of ions and ionophores on cystine egress from human leucocyte lysosome-rich granular fraction.离子和离子载体对人富含白细胞溶酶体颗粒组分中胱氨酸释放的影响。

Biochim Biophys Acta. 1984 Nov 7;777(2):267-73. doi: 10.1016/0005-2736(84)90429-2.

Cysteamine depletes cystinotic leucocyte granular fractions of cystine by the mechanism of disulphide interchange.半胱胺通过二硫键交换机制消耗胱氨酸病白细胞颗粒部分中的胱氨酸。

Biochem J. 1985 Jun 15;228(3):545-50. doi: 10.1042/bj2280545.

Cystine transport is defective in isolated leukocyte lysosomes from patients with cystinosis.胱氨酸病患者分离出的白细胞溶酶体中胱氨酸转运存在缺陷。

Science. 1982 Sep 24;217(4566):1263-5. doi: 10.1126/science.7112129.

Cystinosis phenotypes have identical defective cystine clearance pattern.胱氨酸病的表型具有相同的缺陷性胱氨酸清除模式。

Biomed Pharmacother. 1987;41(9-10):473-7.

Cystine accumulation and loss in normal, heterozygous, and cystinotic fibroblasts.正常、杂合及胱氨酸病成纤维细胞中的胱氨酸积累与损失

Proc Natl Acad Sci U S A. 1982 Jul;79(14):4442-5. doi: 10.1073/pnas.79.14.4442.

Lysosomal cystine counter-transport in heterozygotes for cystinosis.胱氨酸病杂合子中的溶酶体胱氨酸逆向转运

Am J Hum Genet. 1984 Mar;36(2):277-82.

Lysosomal cystine storage in cystinosis and mucolipidosis type II.胱氨酸病和II型粘脂贮积症中的溶酶体胱氨酸贮积

Pediatr Res. 1985 Nov;19(11):1170-4. doi: 10.1203/00006450-198511000-00011.

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In Vitro and In Vivo Models to Study Nephropathic Cystinosis.研究肾性胱氨酸病的体外和体内模型。

Cells. 2021 Dec 21;11(1):6. doi: 10.3390/cells11010006.

Testicular function in males with infantile nephropathic cystinosis.男性婴儿型胱氨酸病的睾丸功能。

Hum Reprod. 2021 Apr 20;36(5):1191-1204. doi: 10.1093/humrep/deab030.

The novel aminoglycoside, ELX-02, permits CTNSW138X translational read-through and restores lysosomal cystine efflux in cystinosis.新型氨基糖苷类药物 ELX-02 可实现 CTNSW138X 的翻译通读，并恢复胱氨酸贮积症中的溶酶体胱氨酸外排。

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Nephropathic Cystinosis: Symptoms, Treatment, and Perspectives of a Systemic Disease.肾性胱氨酸病：一种全身性疾病的症状、治疗及展望

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Lysosomal cystine accumulation promotes mitochondrial depolarization and induction of redox-sensitive genes in human kidney proximal tubular cells.溶酶体胱氨酸积累促进人肾近端小管细胞中的线粒体去极化和氧化还原敏感基因的诱导。

J Physiol. 2016 Jun 15;594(12):3353-70. doi: 10.1113/JP271858. Epub 2016 Apr 10.

Cystine accumulation attenuates insulin release from the pancreatic β-cell due to elevated oxidative stress and decreased ATP levels.由于氧化应激升高和三磷酸腺苷（ATP）水平降低，胱氨酸积累会减弱胰腺β细胞释放胰岛素的能力。

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