نتایج جستجو برای: pseudohypoaldosteronism type 1

تعداد نتایج: 3647227  

اخی, عذرا, بهار, عادله, مخلوق, عطیه, کاشی, زهرا,

Gordon syndrome is a familial autosomal dominant syndrome with hyperkalemia, increased extracellular volume, hypertension, normal kidney function, and metabolic acidosis. It is also called type II Pseudohypoaldosteronism due to usually normal aldosterone and low renin levels. In this report a 35 years old woman is presented with 2-year history of hypertension, eclampsia, and fetal death. Labora...

Journal: :The Biochemical journal 1998
L S Prince M J Welsh

The epithelial Na+ channel (ENaC) complex is composed of three homologous subunits: alpha, beta and gamma. Mutations in ENaC subunits can increase the number of channels on the cell surface, causing a hereditary form of hypertension called Liddle's syndrome, or can decrease channel activity, causing pseudohypoaldosteronism type I, a salt-wasting disease of infancy. To investigate surface expres...

2015
Nina Boiko Volodymyr Kucher James D Stockand

These studies test whether three disease-causing mutations in genes (SCNN1A and SCNN1G) encoding subunits of the epithelial Na(+) channel, ENaC, affect the biophysical and gating properties of this important renal ion channel. The S562P missense mutation in αENaC and the K106_S108delinsN mutation in γENaC are associated with pseudohypoaldosteronism type 1 (PHA1). The N530S missense mutation in ...

Journal: :iranian journal of nursing and midwifery research 0
hamid reza roohafza zahra abdeyazdan parvaneh amini saied pahlavanzadeh pedram shokouh

abstract background: an association of eating disorder with diabetes mellitus may lead to a serious lack of metabolic control, higher mortality and morbidity. there is no recent study conducted in the iranian population about eating disorder and its variants. the aim of the present study is investigation of frequency of disturbed eating behaviors in adolescent girls with type 1 diabetes mellitu...

Journal: :Human molecular genetics 2014
Koichiro Susa Eisei Sohara Tatemitsu Rai Moko Zeniya Yutaro Mori Takayasu Mori Motoko Chiga Naohiro Nomura Hidenori Nishida Daiei Takahashi Kiyoshi Isobe Yuichi Inoue Kenta Takeishi Naoki Takeda Sei Sasaki Shinichi Uchida

Pseudohypoaldosteronism type II (PHAII) is a hereditary disease characterized by salt-sensitive hypertension, hyperkalemia and metabolic acidosis, and genes encoding with-no-lysine kinase 1 (WNK1) and WNK4 kinases are known to be responsible. Recently, Kelch-like 3 (KLHL3) and Cullin3, components of KLHL3-Cullin3 E3 ligase, were newly identified as responsible for PHAII. We have reported that W...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2009
Pedro San-Cristobal Diana Pacheco-Alvarez Ciaran Richardson Aaron M Ring Norma Vazquez Fatema H Rafiqi Divya Chari Kristopher T Kahle Qiang Leng Norma A Bobadilla Steven C Hebert Dario R Alessi Richard P Lifton Gerardo Gamba

Mutations in the kinase WNK4 cause pseudohypoaldosteronism type II (PHAII), a syndrome featuring hypertension and high serum K(+) levels (hyperkalemia). WNK4 has distinct functional states that regulate the balance between renal salt reabsorption and K(+) secretion by modulating the activities of renal transporters and channels, including the Na-Cl cotransporter NCC and the K(+) channel ROMK. W...

Journal: :The Journal of clinical endocrinology and metabolism 2000
T Tajima H Kitagawa S Yokoya K Tachibana M Adachi J Nakae S Suwa S Katoh K Fujieda

Pseudohypoaldosteronism type 1 (PHA1) is a rare condition characterized by neonatal salt loss with dehydration, hypotension, hyperkalemia, and metabolic acidosis, despite elevated plasma aldosterone levels and PRA. Two modes of inheritance of PHA1 have been described: an autosomal dominant form and an autosomal recessive form. An autosomal recessive form manifests severe life-long salt wasting ...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 1997
E Hummler P Barker C Talbot Q Wang C Verdumo B Grubb J Gatzy M Burnier J D Horisberger F Beermann R Boucher B C Rossier

Aldosterone-dependent epithelial sodium transport in the distal nephron is mediated by the absorption of sodium through the highly selective, amiloride-sensitive epithelial sodium channel (ENaC) made of three homologous subunits (alpha, beta, and gamma). In human, autosomal recessive mutations of alpha, beta, or gammaENaC subunits cause pseudohypoaldosteronism type 1 (PHA-1), a renal salt-wasti...

2013
Atsushi Sato Hiroshi Shibuya

WNK kinase family is conserved among many species and regulates SPAK/OSR1 and ion co-transporters. Some mutations in human WNK1 or WNK4 are associated with Pseudohypoaldosteronism type II, a form of hypertension. WNK is also involved in developmental and cellular processes, but the molecular mechanisms underlying its regulation in these processes remain unknown. Here, we identify a new target g...

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