نتایج جستجو برای: pkd1 gene

تعداد نتایج: 1141754  

Ali Hossein Saberi, Atousa Hafizi, Gholamreza Shariati, Hamid Galehdari, Mohammad Hamid, Saeid Reza Khatami,

Introduction: Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common genetic kidney disorders with the incidence of 1 in 1,000 births. ADPKD is genetically heterogeneous with two genes identified: PKD1 (16p13.3, 46 exons) and PKD2 (4q21, 15 exons). Eighty five percent of the patients with ADPKD have at least one mutation in the PKD1 gene. Genetic studies have demonstrate...

Journal: :Human molecular genetics 2007
Irma S Lantinga-van Leeuwen Wouter N Leonhard Annemieke van der Wal Martijn H Breuning Emile de Heer Dorien J M Peters

Autosomal dominant polycystic kidney disease, caused by mutations in the PKD1 gene, is characterized by progressive deterioration of kidney function due to the formation of thousands of cysts leading to kidney failure in mid-life or later. How cysts develop and grow is currently unknown, although extensive research revealed a plethora of cellular changes in cyst lining cells. We have constructe...

Journal: :BioTechniques 1999
W Thongnoppakhun P Wilairat K Vareesangthip P T Yenchitsomanus

Characterization of mutations of the PKD1 gene has been limited by the fact that three-fourths of this gene at its 5' end is homologous to sequences of at least three other genes on the same chromosome. We have therefore developed a method of long reverse transcription PCR for selective amplification of the entire coding sequence of the PKD1 gene from its mRNA. A PCR primer specific to the sequ...

Journal: :Human molecular genetics 1997
T J Watnick K B Piontek T M Cordal H Weber M A Gandolph F Qian X M Lens H P Neumann G G Germino

The gene for the most common and severe form of autosomal dominant polycystic kidney disease, PKD1, encodes a 14 kb mRNA that is predicted to result in an integral membrane protein of 4302 amino acids. The major challenge faced by researchers attempting to complete mutation analysis of the PKD1 gene has been the presence of several homologous loci also located on chromosome 16. Because the sequ...

Journal: :Journal of immunology 2008
Jeoung-Eun Park Young-In Kim Ae-Kyung Yi

Protein kinase D1 (PKD1) is expressed ubiquitously and regulates diverse cellular processes such as oxidative stress, gene expression, cell survival, and vesicle trafficking. However, the presence and function of PKD1 in monocytic cells are currently unknown. In this study, we provide evidence that PKD1 is involved in TLR9 signaling in macrophages. Class B-type CpG DNA (CpG-B DNA) induced activ...

Journal: :Cell 1995
The International Polycystic Kidney Disease Consortium

Mutations in the PKD1 gene are the most common cause of autosomal dominant polycystic kidney disease (ADPKD). Other PKD1-like loci on chromosome 16 are approximately 97% identical to PKD1. To determine the authentic PKD1 sequence, we obtained the genomic sequence of the PKD1 locus and assembled a PKD1 transcript from the sequence of 46 exons. The 14.5 kb PKD1 transcript encodes a 4304 amino aci...

2013
Jana Reiterová Jitka Štekrová Miroslav Merta Jaroslav Kotlas Veronika Elišáková Petr Lněnička Marie Korabečná Milada Kohoutová Vladimír Tesař

BACKGROUND Autosomal dominant polycystic kidney disease (ADPKD) is the most common form of inherited kidney disease that results in renal failure. ADPKD is a systemic disorder with cysts and connective tissue abnormalities involving many organs. ADPKD caused by mutations in PKD1 gene is significantly more severe than the cases caused by PKD2 gene mutations. The large intra-familial variability ...

Journal: :Journal of the American Society of Nephrology : JASN 2006
Peter C Harris Kyongtae T Bae Sandro Rossetti Vicente E Torres Jared J Grantham Arlene B Chapman Lisa M Guay-Woodford Bernard F King Louis H Wetzel Deborah A Baumgarten Philip J Kenney Mark Consugar Saulo Klahr William M Bennett Catherine M Meyers Qin Jean Zhang Paul A Thompson Fang Zhu J Philip Miller

Data from serial renal magnetic resonance imaging of the Consortium of Radiologic Imaging Study of PKD (CRISP) autosomal dominant polycystic kidney disease (PKD) population showed that cystic expansion occurs at a consistent rate per individual, although it is heterogeneous in the population, and that larger kidneys are associated with more rapid disease progression. The significance of gene ty...

2017
Susana Franco Santos Telma Francisco Ana Isabel Cordeiro Maria João Paiva Lopes

Tuberous sclerosis(TS) is an autosomal dominant disease caused by mutations in TSC1 and TSC2 genes. TSC2 gene is located in chromosome 16p13.3, adjacent to PKD1 gene, responsible for the autosomal dominant polycystic kidney disease. In a rare subgroup of patients, the presence of a deletion which simultaneously affects the TSC2 and PKD1 genes has been confirmed. TSC2/PKD1-Contiguous Gene Syndro...

Journal: :Journal of medical genetics 1990
M H Breuning F G Snijdewint H Brunner A Verwest J W Ijdo J J Saris J G Dauwerse L Blonden T Keith D F Callen

To define the PKD1 locus further, the gene involved in the most frequent form of adult polycystic kidney disease, probes from 16 polymorphic loci were mapped on 16p13.1-pter with the combined use of cell lines containing rearranged chromosomes and family studies. Five breakpoints in the distal part of 16p arbitrarily subdivided the loci into five groups. By analysing 58 recombination events amo...

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