نتایج جستجو برای: npc2

تعداد نتایج: 199  

2014
Lisette J. A. Kogelman Sameer D. Pant Merete Fredholm Haja N. Kadarmideen

Obesity is a complex condition with world-wide exponentially rising prevalence rates, linked with severe diseases like Type 2 Diabetes. Economic and welfare consequences have led to a raised interest in a better understanding of the biological and genetic background. To date, whole genome investigations focusing on single genetic variants have achieved limited success, and the importance of inc...

2007
Marc C. Patterson Marie T. Vanier Kinuko Suzuki Jill A. Morris Eugene Carstea Edward B. Neufeld Joan E. Blanchette-Mackie Peter G. Pentchev

1. Niemann-Pick disease type C (NP-C) is an autosomal recessive lipidosis with protean clinical manifestations, distinguished biochemically by a unique error in cellular trafficking of exogenous cholesterol that is associated with lysosomal accumulation of unesterified cholesterol. A majority of patients with this phenotype are linked genetically to chromosome 18, the locus of Niemann-Pick dise...

2005
Veena D. Dwivedi Natalie A. Phillips Maude Laguë-Beauvais Shari R. Baum

The goal of this paper is to explore an on-line processing theory of discourse comprehension which makes use of the grammatical construct of modality. Previous investigations of discourse processing have relied upon studies using the integrative device of anaphora (Halliday and Hasan, 1976). A close examination of the kinds of discourses previously investigated reveals that the sorts of inferen...

2017
Yohan Choi Abdolreza Hosseindoust YoungHo Shim Minju Kim Alip Kumar Seungmin Oh YoungHwa Kim Byung-Jo Chae

OBJECTIVE The present study investigated the litter performance of multiparous sows fed 3% and 6% densified diets at farrowing to weaning during summer with mean maximum room temperature of 30.5°C. METHODS A total of 60 crossbred multiparous sows were allotted to one of three treatments based on body weight according to a completely randomized design. Three different nutrient levels based on ...

2016
Max Dougherty John Lazar Jason C. Klein Karina Diaz Theodore Gobillot Eli Grunblatt Nicholas Hasle Daniel Lawrence Megan Maurano Maria Nelson Gregory Olson Sanjay Srivatsan Jay Shendure C. Dirk Keene Thomas Bird Marshall S. Horwitz Desiree A. Marshall

Adult-onset Niemann-Pick disease type C (NPC) is an infrequent presentation of a rare neurovisceral lysosomal lipid storage disorder caused by autosomal recessive mutations in NPC1 (∼95%) or NPC2 (∼5%). Our patient was diagnosed at age 33 when he presented with a 10-yr history of difficulties in judgment, concentration, speech, and coordination. A history of transient neonatal jaundice and sple...

2017
Keiichi Motoyama Rena Nishiyama Yuki Maeda Taishi Higashi Yoichi Ishitsuka Yuki Kondo Tetsumi Irie Takumi Era Hidetoshi Arima

Niemann-Pick type C (NPC) disease, characterized by intracellular accumulation of unesterified cholesterol and other lipids owing to defects in two proteins NPC1 and NPC2, causes neurodegeneration and other fatal neurovisceral symptoms. Currently, treatment of NPC involves the use of 2-hydroxypropyl-β-cyclodextrin (HP-β-CD). HP-β-CD is effective in the treatment of hepatosplenomegaly in NPC dis...

Journal: :EBioMedicine 2016
Xuntian Jiang Daniel S Ory

Niemann–Pick C (NPC) disease is rare, neurodegenerative, lysosomal cholesterol storage disorder. Diagnosis of the disease is often delayed due to disease heterogeneity, non-specific early visceral and neurological symptoms, and lack of a rapid and reliable diagnostic assay. As a result, the disease progresses and opportunities to intervene tragically are lost. Until recently, the principal diag...

Journal: :Molecules 2015
Masego Dibetsoe Lukman O Olasunkanmi Omolola E Fayemi Sasikumar Yesudass Baskar Ramaganthan Indra Bahadur Abolanle S Adekunle Mwadham M Kabanda Eno E Ebenso

The effects of seven macrocyclic compounds comprising four phthalocyanines (Pcs) namely 1,4,8,11,15,18,22,25-octabutoxy-29H,31H-phthalocyanine (Pc1), 2,3,9,10,16,17,23,24-octakis(octyloxy)-29H,31H-phthalocyanine (Pc2), 2,9,16,23-tetra-tert-butyl-29H,31H-phthalocyanine (Pc3) and 29H,31H-phthalocyanine (Pc4), and three naphthalocyanines namely 5,9,14,18,23,27,32,36-octabutoxy-2,3-naphthalocyanine...

Journal: :PloS one 2016
Elena-Raluca Nicoli Nada Al Eisa Celine V M Cluzeau Christopher A Wassif James Gray Kathryn R Burkert David A Smith Lauren Morris Stephanie M Cologna Cody J Peer Tristan M Sissung Constantin-Daniel Uscatu William D Figg William J Pavan Charles H Vite Forbes D Porter Frances M Platt

Niemann-Pick type C (NPC) disease is a neurodegenerative lysosomal storage disease caused by mutations in either the NPC1 or NPC2 gene. NPC is characterised by storage of multiple lipids in the late endosomal/lysosomal compartment, resulting in cellular and organ system dysfunction. The underlying molecular mechanisms that lead to the range of clinical presentations in NPC are not fully underst...

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