کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
4012434 | 1261194 | 2007 | 5 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
A dual Golgi- and mitochondria-localised Ala25Ser precursor cystatin C: An additional tool for characterising intracellular mis-localisation leading to increased AMD susceptibility
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موضوعات مرتبط
علوم زیستی و بیوفناوری
ایمنی شناسی و میکروب شناسی
ایمونولوژی و میکروب شناسی (عمومی)
پیش نمایش صفحه اول مقاله
![عکس صفحه اول مقاله: A dual Golgi- and mitochondria-localised Ala25Ser precursor cystatin C: An additional tool for characterising intracellular mis-localisation leading to increased AMD susceptibility A dual Golgi- and mitochondria-localised Ala25Ser precursor cystatin C: An additional tool for characterising intracellular mis-localisation leading to increased AMD susceptibility](/preview/png/4012434.png)
چکیده انگلیسی
An artificial mutant Ala25Ser precursor cystatin C was created to help elucidate the cause of intracellular mis-localisation of the biochemically related variant B (Ala25Thr) precursor cystatin C to the mitochondria. Homozygotes of variant B precursor cystatin C were reported to carry an increased susceptibility to developing the exudative form of AMD. Ala25Ser precursor cystatin C shows a dual distribution to the Golgi apparatus and to the mitochondria. This localisation is thus intermediary between that of wild-type cystatin C (targeted to ER/Golgi compartment) and that of variant B precursor cystatin C. Furthermore, the level of secretion of Ala25Ser cystatin C by RPE cells is intermediary between wild type and variant B cystatin C. Ala25Ser precursor cystatin C thus represents a biochemical intermediate between the wild type and the AMD-associated cystatin C and as such, is a novel tool for the investigation of the mechanism of intracellular mis-localisation of variant B cystatin C. Our findings further support the hypothesis that substitution of the alanine residue in the penultimate position of precursor cystatin C signal sequence with a less hydrophobic amino acid residue, such as threonine (as in variant B cystatin C) or serine is sufficient to impair the intracellular trafficking and processing of the protein.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Experimental Eye Research - Volume 84, Issue 6, June 2007, Pages 1135-1139
Journal: Experimental Eye Research - Volume 84, Issue 6, June 2007, Pages 1135-1139
نویسندگان
Arjuna Ratnayaka, Luminita Paraoan, Dave G. Spiller, Paul Hiscott, Glyn Nelson, Michael R.H. White, Ian Grierson,