نتایج جستجو برای: tuberous sclerosis complex tsc

تعداد نتایج: 845362  

2014
Emily A. Greene-Colozzi Abbey R. Sadowski Elyza Chadwick Peter T. Tsai Mustafa Sahin

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by tumor growth and neuropsychological symptoms such as autistic behavior, developmental delay, and epilepsy. While research has shed light on the biochemical and genetic etiology of TSC, the pathogenesis of the neurologic and behavioral manifestations remains poorly understood. TSC patients have a greatly increase...

Journal: :FEBS letters 2006
Min Wan Xiaohui Wu Kun-Liang Guan Min Han Yuan Zhuang Tian Xu

Muscle mass is regulated by a wide range of hormonal and nutritional signals, such as insulin and IGF. Tuberous sclerosis complex (TSC) is an inherited hamartoma disease with tumor growth in numerous organs. TSC is caused by mutation in either TSC1 or TSC2 tumor suppressor genes that negatively regulate insulin-induced S6K activation and cell growth. Here we report that expression of human TSC1...

Cardiac rhabdomyomas are the most common primary cardiac tumors in children. These tumors are generally asymptomatic, although they may be associated with neonatal tuberous sclerosis complex. Despite the fact that thyroid dysfunction rarely occurs in tuberous sclerosis, papillary adenomas (hamartomas) of the thyroid gland have been reported in a number of autopsies. Herein, we present the case ...

Journal: :Journal of autism and developmental disorders 1992
S L Smalley P E Tanguay M Smith G Gutierrez

Autism is a behavior disorder with genetic influences indicated from twin and family studies and from the co-occurrence of autism with known genetic disorders. Tuberous sclerosis complex (TSC) is a known genetic disorder with behavioral manifestations including autism. A literature review of these two disorders substantiates a significant association of autism and TSC with 17-58% of TSC subject...

Journal: :Human mutation 2016
Rosemary Ekong Mark Nellist Marianne Hoogeveen-Westerveld Marjolein Wentink Jessica Panzer Steven Sparagana Warren Emmett Natalie L Dawson Marie Claire Malinge Rima Nabbout Caterina Carbonara Marco Barberis Sergio Padovan Marta Futema Vincent Plagnol Steve E Humphries Nicola Migone Sue Povey

Inactivating mutations in TSC1 and TSC2 cause tuberous sclerosis complex (TSC). The 2012 international consensus meeting on TSC diagnosis and management agreed that the identification of a pathogenic TSC1 or TSC2 variant establishes a diagnosis of TSC, even in the absence of clinical signs. However, exons 25 and 31 of TSC2 are subject to alternative splicing. No variants causing clinically diag...

Journal: :Frontiers in Neurology 2021

Background: Epilepsy is the most common neurological manifestation in individuals with tuberous sclerosis complex (TSC). However, real-world evidence on diagnosis and treatment patterns limited. Here, we present data from TuberOus Sclerosis registry to increase disease Awareness (TOSCA) changes of epilepsy diagnosis, treatments, outcomes over time, detailed characteristics substudy. Methods: wa...

Journal: :Molecular and cellular biology 2005
Avi Sofer Kui Lei Cory M Johannessen Leif W Ellisen

The tuberous sclerosis tumor suppressors TSC1 and TSC2 regulate the mTOR pathway to control translation and cell growth in response to nutrient and growth factor stimuli. We have recently identified the stress response REDD1 gene as a mediator of tuberous sclerosis complex (TSC)-dependent mTOR regulation by hypoxia. Here, we demonstrate that REDD1 inhibits mTOR function to control cell growth i...

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