Jared W Benedict
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Explore the profile of Jared W Benedict including associated specialties, affiliations and a list of published articles.
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9
Citations
241
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Recent Articles
1.
Getty A, Benedict J, Pearce D
Exp Cell Res
. 2010 Sep;
317(1):51-69.
PMID: 20850431
Juvenile neuronal ceroid lipofuscinosis (JNCL) is a pediatric lysosomal storage disorder characterized by accumulation of autofluorescent storage material and neurodegeneration, which result from mutations in CLN3. The function of CLN3,...
2.
Vitiello S, Benedict J, Padilla-Lopez S, Pearce D
Hum Mol Genet
. 2009 Dec;
19(5):931-42.
PMID: 20015955
Juvenile Batten disease is an autosomal recessive pediatric neurodegenerative disorder caused by mutations in the CLN3 gene. The CLN3 protein primarily resides in the lysosomal membrane, but its function is...
3.
Benedict J, Getty A, Wishart T, Gillingwater T, Pearce D
J Neurosci Res
. 2009 Feb;
87(9):2157-66.
PMID: 19235893
Mutations in CLN6 cause variant late-onset neuronal ceroid lipofuscinosis (vLINCL), a childhood neurodegenerative disorder resulting from aberrant neuronal cell loss and pathological accumulation of lysosomal autofluorescent storage material in the...
4.
Weimer J, Benedict J, Getty A, Pontikis C, Lim M, Cooper J, et al.
Brain Res
. 2009 Feb;
1266:93-107.
PMID: 19230832
Juvenile neuronal ceroid lipofuscinosis (JNCL), or Batten disease, is a neurodegenerative disease resulting from a mutation in CLN3, which presents clinically with visual deterioration, seizures, motor impairments, cognitive decline, hallucinations,...
5.
Benedict J, Sommers C, Pearce D
J Neurosci Res
. 2007 Jul;
85(13):2882-91.
PMID: 17638298
Oxidative damage is a known contributor to the pathogenesis of neurodegenerative diseases. Juvenile Batten disease is a progressive neurodegenerative disorder of childhood that results from mutation in Cln3. We have...
6.
Weimer J, Benedict J, Elshatory Y, Short D, Ramirez-Montealegre D, Ryan D, et al.
Brain Res
. 2007 Jul;
1162:98-112.
PMID: 17617387
Batten disease, or juvenile neuronal ceroid lipofuscinosis (JNCL), results from mutations in the CLN3 gene. This disorder presents clinically around the age of 5 years with visual deficits progressing to...
7.
Lim M, Alexander N, Benedict J, Chattopadhyay S, Shemilt S, Guerin C, et al.
Neurobiol Dis
. 2006 Oct;
25(2):239-51.
PMID: 17070688
Patients and a mouse model of Batten disease, the juvenile form of neuronal ceroid lipofuscinosis (JNCL), raise autoantibodies against GAD65 and other brain-directed antigens. Here we investigate the adaptive component...
8.
Weimer J, Custer A, Benedict J, Alexander N, Kingsley E, Federoff H, et al.
Neurobiol Dis
. 2006 Jan;
22(2):284-93.
PMID: 16412658
Juvenile neuronal ceroid lipofuscinosis (JNCL) is an autosomal recessive disorder of childhood caused by mutations in CLN3. Although visual deterioration is typically the first clinical sign to manifest in affected...
9.
Phillips S, Benedict J, Weimer J, Pearce D
J Neurosci Res
. 2005 Jan;
79(5):573-83.
PMID: 15657902
Batten disease, an inherited neurodegenerative storage disease affecting children, results from the autosomal recessive inheritance of mutations in Cln3. The function of the CLN3 protein remains unknown. A key to...