Focal segmental glomerular sclerosis, a type of intractable chronic glomerulonephritis, is a stem cell disorder
نویسندگان
چکیده
The etiopathogenesis of focal and segmental glomerular sclerosis (FGS) remains unknown. Using a new animal model for FGS (FGS mouse), we demonstrate here that bone marrow transplantation from normal mice to FGS mice with a high grade of proteinuria (+ + +) ameliorates FGS, and that the transplantation of bone marrow cells or purified hemopoietic stem cells (HSCs) from FGS mice induces FGS in normal mice. These findings strongly suggest that FGS is a stem cell disorder; the abnormalities may be genetically programmed at the level of HSCs.
منابع مشابه
Cyclosporin A in refractory idiopathic nephrotic syndrome: 5 years clinical experience.
The use of cyclosporin A (Cy A) in idiopathic nephrotic syndrome, particularly lesions of focal segmental glomerular sclerosis, is controversial. A retrospective study of 10 adult patients with nephrotic syndrome treated with Cy A was performed. Histological diagnosis was established in all patients: focal segmental glomerular sclerosis (n = 6), focal global sclerosis (n = 1), mesangial prolife...
متن کاملRole of parietal epithelial cells in kidney injury: the case of rapidly progressing glomerulonephritis and focal and segmental glomerulosclerosis.
BACKGROUND Millions of people are affected by irreversible loss of renal function and thus by a significantly increased cardiovascular risk. In this context, the parietal epithelial cells (PECs) of the glomerulus have attracted increasing attention in recent years. So far, they have been ascribed 2 major functions: (1) PECs may act as intrinsic progenitor cells to replenish podocytes and/or pro...
متن کاملIgA localisation in glomerular diseases.
The structural changes found on light and electron microscopy study of 25 renal biopsy specimens that showed significant glomerular IgA deposition on immunofluorescence were correlated with relevant clinical data. The morphology of a wide range of glomerulopathies seeen included mesangial proliferative (60%), membranous (12%), rapidly progressive proliferative (8%), mesangio-capillary (8%), and...
متن کاملA clinicopathologic study of focal segmental glomerulosclerosis: comparison between nephrotic and non-nephrotic focal segmental glomerulosclerosis.
Seven out of 16 patients with primary focal segmental glomerulosclerosis (FGS) did not showed the nephrotic syndrome throughout their clinical courses, and then patients with FGS could be divided into the two groups, nephrotic FGS group (NS-G) and non-nephrotic one (NO-G). The clinicopathologic findings of NS-G and NO-G were compared retrospectively to define the pathogenesis of the glomerular ...
متن کاملA novel heterozygous COL4A4 missense mutation in a Chinese family with focal segmental glomerulosclerosis
Focal segmental glomerulosclerosis (FSGS) is the most common glomerular histological lesion associated with high-grade proteinuria and end-stage renal disease. Histologically, FSGS is characterized by focal segmental sclerosis with foot process effacement. The aim of this study was to identify the disease-causing mutation in a four-generation Chinese family with FSGS. A novel missense mutation,...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- The Journal of Experimental Medicine
دوره 179 شماره
صفحات -
تاریخ انتشار 1994