نتایج جستجو برای: gdap1

تعداد نتایج: 91  

2018
Xin Zhao Ming-Ming Jiang Yi-Zhou Yan Lei Liu Yong-Zhi Xie Xiao-Bo Li Zheng-Mao Hu Xiao-Hong Zi Kun Xia Bei-Sha Tang Ru-Xu Zhang

BACKGROUND SH3TC2, PMP2, and BSCL2 genes are related to autosomal recessive (AR) Charcot-Marie-Tooth (CMT) disease type 1, autosomal dominant (AD)-CMT1, and AD-CMT2, respectively. Pathogenic variants in these three genes were not well documented in Chinese CMT patients. Therefore, this study aims to detect SH3TC2, PMP2, and BSCL2 pathogenic variants in a cohort of 315 unrelated Chinese CMT fami...

2014
Kunzhe Dong Na Yao Yabin Pu Xiaohong He Qianjun Zhao Yizhao Luan Weijun Guan Shaoqi Rao Yuehui Ma Marinus FW. te Pas

High altitude environments are of particular interest in the studies of local adaptation as well as their implications in physiology and clinical medicine in human. Some Chinese pig breeds, such as Tibetan pig (TBP) that is well adapted to the high altitude and Dahe pig (DHP) that dwells at the moderate altitude, provide ideal materials to study local adaptation to altitudes. Yet, it is still s...

2016
Celeste Montecchiani Lucia Pedace Temistocle Lo Giudice Antonella Casella Marzia Mearini Fabrizio Gaudiello José L. Pedroso Chiara Terracciano Carlo Caltagirone Roberto Massa Peter H. St George-Hyslop Orlando G. P. Barsottini Toshitaka Kawarai Antonio Orlacchio

Charcot-Marie-Tooth disease is a group of hereditary peripheral neuropathies that share clinical characteristics of progressive distal muscle weakness and atrophy, foot deformities, distal sensory loss, as well as diminished tendon reflexes. Hundreds of causative DNA changes have been found, but much of the genetic basis of the disease is still unexplained. Mutations in the ALS5/SPG11/KIAA1840 ...

Journal: :genetics in the 3rd millennium 0
نادر لطفعلی زاده  nader lotfalizadeh allameh amini genetics counceling center of social welfare, 17 shahrivar st, tabriz, iranتبریز، خیابان هفده شهریور جدید، مرکز مشاوره علامه امینی بهزیستی معصومه جنت دوست masoumeh janat doust فرحناز ریحانی فر farahnaz reyhanifar نوشین سرخکوه آذری noushin sorkhkoh azari مجید رضائی بصیری majid rezai-basiri شیوا ثقفی shiva saghafi صدیقه نوبخت

diseases of the motor unit are common in children. these diseases are mostly genetically determined. cmt represents a clinically heterogeneous group of disorders caused by aberration of the intimate relationship between the schwann cell sheath and the neural axon, ultimately resulting in axonal death and muscular dennervation. a simple clinical classification of cmt (demyelinating versus axonal...

Journal: :Journal of medical genetics 2005
R Claramunt L Pedrola T Sevilla A López de Munain J Berciano A Cuesta B Sánchez-Navarro J M Millán G M Saifi J R Lupski J J Vílchez C Espinós F Palau

C harcot-Marie-Tooth (CMT) disease is a motor and sensory neuropathy with clinical and genetic heterogeneity. Patients usually present in the first or second decade of life with distal muscle atrophy in the legs, areflexia, foot deformity (mainly pes cavus), and steppage gait. In most cases, hands are also involved as the disease progresses. CMT is the most frequent inherited neuropathy, with a...

2006
Jung-Hwa Lee Byung-Ok Choi

Charcot-Marie-Tooth disease (CMT) is the most common form of inherited motor and sensory neuropathy. Moreover, CMT is a genetically heterogeneous disorder of the peripheral nervous system, with many genes identified as CMT-causative. CMT has two usual classifications: type 1, the demyelinating form (CMT1); and type 2, the axonal form (CMT2). In addition, patients are classified as CMTX if they ...

Journal: :genetics in the 3rd millennium 0
سید محمد حسن تنکابنی mohammad hassan tonekaboni pediatric neurologist, associate professor of shaheed beheshti medical university, mofid children s hospital.

according to the well-known classification of dyck, inherited peripheral neuropathies can be categorized as hereditary motor and sensory neuropathies (hmsn) or charcot – marie – tooth (cmt) disease, hereditary motor neuropathies (hmn), and hereditary sensory neuropathies (hsn). cmt is a clinically and genetically heterogeneous group of motor and sensory neuropathies, and is the most common inhe...

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