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Elsevier电子期刊(64)
在“
Elsevier电子期刊
”中,
命中:
64
条,耗时:小于0.01 秒
在所有数据库中总计命中:
64
条
1.
Astrocytes and lysosomal storage diseases
作者:
K.V. Rama Rao
;
T. Kielian
;
tkielian@unmc.edu" class="auth_mail" title="E-mail the corresponding author
关键词:
AD
;
Alzheimer&rsquo
;
s disease
;
DAMP
;
danger-associated molecular pattern
;
GBA
;
glucocerebrosidase
;
GFAP
;
glial fibrillary acidic protein
;
GLD
;
globoid cell leukodystrophy
;
INCL
;
infantile neuronal ceroid lipofuscinosis
;
JNCL
;
juvenile neuronal ceroid lipofuscinosis
;
LINCL
;
late infantile neuronal ceroid lipofuscinosis
;
LMP
;
lysosomal membrane permeability
;
LSD
;
lysosomal storage disease
;
MSD
;
multiple sulfatase deficiency
;
NCL
;
neuronal ceroid lipofuscinosis
;
NPC
;
Niemann-Pick type C
;
PD
;
Parkin
刊名:Neuroscience
出版年:2016
2.
A novel mutation af Cln3 associated with delayed-classic juvenile ceroid lipofuscinois and autophagic vacuolar myopathy
作者:
L. Licchetta
a
;
b
;
laura.licchetta2@unibo.it" class="auth_mail" title="E-mail the corresponding author
;
F. Bisulli
a
;
b
;
M. Fietz
c
;
M.L. Valentino
a
;
b
;
M. Morbin
d
;
B. Mostacci
a
;
K.L. Oliver
e
;
S.F. Berkovic
e
;
P. Tinuper
a
;
b
关键词:
Juvenile neuronal ceroid lipofuscinosis (
JNCL
)
;
Delayed-classic
JNCL
;
CLN3
;
Autophagic vacuolar myopathy
刊名:European Journal of Medical Genetics
出版年:2015
3.
Antigen presenting cell abnormalities in the Cln3
−/−
mouse model of juvenile neuronal ceroid lipofuscinosis
作者:
Samantha L. Hersrud
;
Attila D. Ková
;
cs
;
David A. Pearce
;
david.pearce@sanfordhealth.org" class="auth_mail" title="E-mail the corresponding author
关键词:
APCs
;
antigen presenting cells
;
APhC
;
allophycocyanin
;
BM
;
bone marrow
;
BMCCs
;
bone marrow cultured cells
;
BM-DCs
;
bone marrow dendritic cells
;
BM-Mφ
;
bone marrow macrophages
;
BSA
;
bovine serum albumin
;
CHX
;
cycloheximide
;
CTB
;
cholera toxin B
;
DCs
;
dendritic cells
;
DPBS
;
Dulbecco's phosphate buffered saline
;
EDTA
;
ethylenediaminetetraacetic acid
;
FBS
;
fetal bovine serum
;
GM-CSF
;
granulocyte macrophage-colony stimulating factor
;
IFN-γ
;
interferon-γ
;
JNCL
;
juvenile neuronal ceroid lipofuscinosis
刊名:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
出版年:2016
4.
Methodology of clinical research in rare diseases: Development of a research program in juvenile neuronal ceroid lipofuscinosis (
JNCL
) via creation of a patient registry and collaboration with patient advocates
作者:
Elisabeth A. de Blieck
;
Erika F. Augustine
;
Frederick J. Marshall
;
Heather Adams
;
Jennifer Cialone
;
Leon Dure
;
Jennifer M. Kwon
;
Nicole Newhouse
;
Katherine Rose
;
Paul G. Rothberg
;
Amy Vierhile
;
Jonathan W. Mink
关键词:
BDSRA
;
Batten Disease Support and Research Association
;
JNCL
;
juvenile neuronal ceroid lipofuscinosis
;
NCL
;
neuronal ceroid lipofuscinosis
;
NIH
;
National Institutes of Health
;
UBDRS
;
Unified Batten Disease Rating Scale
;
URBC
;
University of Rochester Batte
刊名:Contemporary Clinical Trials
出版年:2013
5.
Bridging NCL research gaps
作者:
Frank Stehr
;
frank.stehr@ncl-stiftung.de" class="auth_mail" title="E-mail the corresponding author
;
Herman van der Putten
关键词:
NCL
;
neuronal ceroid lipofuscinosis
;
LSD
;
lysosomal storage disease
;
ERT
;
enzyme replacement therapy
;
ER
;
endoplasmic reticulum
;
PoC
;
proof-of-concept
;
iPS cell
;
induced pluripotent stem cell
;
PD
;
Parkinson's disease
;
NPC
;
Niemann&ndash
;
Pick type C
;
SLOS
;
Smith&ndash
;
Lemli&ndash
;
Opitz syndrome
;
FTLD
;
frontotemporal lobe degeneration
;
JNCL
;
juvenile NCL
;
EU
;
European Union
;
DEM-CHILD
;
dementia in childhood
;
EEG
;
electroencephalography
;
MRI
;
magnetic resonance imaging
刊名:Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
出版年:2015
6.
Hippocampal Volumes in Juvenile Neuronal Ceroid Lipofuscinosis: A Longitudinal Magnetic Resonance Imaging Study
作者:
Anna M. Tokola
;
Eero K. Salli
;
Laura E. 脜berg
;
Taina H. Autti
关键词:
juvenile neuronal ceroid lipofuscinosis
;
JNCL
;
NCL
;
CLN3
;
Batten disease
;
lysosomal storage disease
;
hippocampus
;
MRI volumetry
刊名:Pediatric Neurology
出版年:February, 2014
7.
The Batten disease gene CLN3 confers resistance to endoplasmic reticulum stress induced by tunicamycin
作者:
Dan Wu
a
;
danw@bjmu.edu.cn" class="auth_mail
;
Jing Liu
a
;
Baiyan Wu
a
;
Bo Tu
b
;
Weiguo Zhu
b
;
Jianyuan Luo
a
;
c
;
jluo@som.umaryland.edu" class="auth_mail
关键词:
CLN3
;
Endoplasmic reticulum (ER) stress
;
JNCL
;
Tunicamycin
刊名:Biochemical and Biophysical Research Communications
出版年:25 April 2014
8.
Altered levels of 伪-synuclein and sphingolipids in Batten disease lymphoblast cells
作者:
Sunyang Kang
a
;
Tae-Hwe Heo
b
;
thhur92@catholic.ac.kr" class="auth_mail
;
Sung-Jo Kim
a
;
sungjo@hoseo.edu" class="auth_mail
关键词:
JNCL
;
juvenile neuronal ceroid lipofuscinosis
;
AD
;
Alzheimer's disease
;
PD
;
Parkinson's disease
;
GD
;
Gaucher's disease
刊名:Gene
出版年:15 April, 2014
9.
Valproate-induced hyperammonemia in juvenile ceroid lipofuscinosis (Batten disease)
作者:
Erling P. Larsen
;
John R. Ø
;
stergaard
;
john.oestergaard@skejby.rm.dk" class="auth_mail
关键词:
Batten disease
;
Carnitine
;
Hyperammonemia
;
Juvenile ceroid lipofuscinosis
;
Valproate
;
Valproate-induced hyperammonemic encephalopathy
刊名:Seizure
出版年:June 2014
10.
Severe sinus node dysfunction in a patient with juvenile neuronal ceroid lipofuscinosis
作者:
Polychronis Dilaveris
;
hrodil1@yahoo.com" class="auth_mail
;
Iosif Koutagiar
;
Constantina Aggeli
;
Skevos Sideris
;
Konstantinos Gatzoulis
;
Christodoulos Stefanadis
关键词:
Sinus node dysfunction
;
JNCL
;
Pacemaker implantation
;
Sinus bradycardia
;
Juvenile neuronal ceroid lipofuscinosis
刊名:International Journal of Cardiology
出版年:1 June 2014
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