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PASCAL is one of the world's leading sources of life and physical
sciences information.
PASCAL is produced by the Institut de l'Information Scientifique
et Technique (INIST) of the French National Research Council
(CNRF).
It provides access to the world's scientific and technical
literature including pure and applied biology, homeopathy,
medicine, botany, psychology, pharmacology, toxicology,
biotechnology, agriculture, physics, chemistry, information
sciences, telecommunications, construction industry, mechanical
engineering, metallurgy, earth sciences, oceanography, and
astronomy. About 500,000 new citations are added to PASC each
year in these diverse disciplines. French and European
literature is particularly well represented in PASCAL. The
database includes references to journal articles, books, reports,
theses and conference proceedings.
Over 80% of documents have an abstract and most have an English
language title. PASCAL is well indexed to aid subject retrieval
in this multi-disciplinary file, several classification schemes
are used and controlled descriptors from a vocabulary of over
80,000 terms are assigned in English, French and Spanish.
Texts from all over the world in 100 different languages are
analysed for inclusion in PASCAL. The coverage in PASC by
language is approximately as follows: English 63%, French 12%,
Russian 10%, German 8%, and other languages 7%. INIST also offers
a free, multilingual and multidisciplinary guide to scientific
terminology at http://www.termsciences.fr
Use PASC to find information on a wide range of topics, including
such matters as:
- what papers have been published by Havemann working in Marburg
University, Germany?
- which methods are used for controlling pests in beet growing,
and which insecticides are currently undergoing field trials?
- what is the effectiveness of diet on controlling diabetes?
- what has been published on quantum mechanical studies on spin?
Sources:
Literature of all types is abstracted in PASCAL: journal articles
(about 93% of the file), theses (mainly French, 5,000 per year),
conference proceedings (6% of the file), technical reports
and books. Over 6,500 journals are scanned regularly and over
4,500 of these are abstracted completely.
Accession number & update
0017097418 20080404.
Title
Purification of neuronal inclusions of patients with
Huntington's disease reveals a broad range of N-terminal
fragments of expanded huntingtin and insoluble polymers.
Source
Journal of neurochemistry, {J-neurochem}, 2005, vol. 95, no.
1, p. 125-136, 12 pp., 1 p.1/4 refs, CODEN: JONRA9, ISSN:
0022-3042.
Publisher: Blackwell, Oxford, United Kingdom.
Author(s)
HOFFNER-Guylaine, ISLAND-Marie-Laure, DJIAN-Philippe.
Author affiliation
UPR 2228 CNRS, Institut Interdisciplinaire des Sciences du
Vivant, Universite Rene Descartes, Paris, France.
Abstract
Huntington's disease resulting from huntingtin containing an
expanded polyglutamine is associated with aggregates largely
confined to neuronal inclusions, and with neuronal death.
Inclusions are thought to originate from discrete N-terminal
fragments of expanded huntingtin produced by specific
endopeptidases. We have now purified the neuronal inclusions
of Huntington's disease brain. When incubated in concentrat-
ed formic acid, purified inclusions release a polymer, an
oligomer and a broad range of N-terminal fragments of expanded
huntingtin. The fragments and the polymeric forms are linked
to each other by non-covalent bonds as they are both released
by formic acid, whereas the polymeric forms themselves are
presumably stabilized by covalent bonds, as they are resistant
to formic acid. We also demonstrate the presence in affected
areas of the brain but not in unaffected areas of a broad
range of soluble N-terminal fragments of expanded huntingtin
not yet associated with the inclusions and which are likely to
be the precursors of the inclusions. Fragmentation of expan-
ded huntingtin in Huntington's disease must result from the
operation of multiple proteolytic activities with little
specificity and not from that of a specific endopeptidase;
subsequent aggregation of the fragments by covalent and non-
covalent bonds leads to the formation of the inclusions.
Descriptors
Nervous-system-diseases; Central-nervous-system-disease;
Genetic-disease; Degenerative-disease; Extrapyramidal-
syndrome; Enzyme; Hydrolases; Peptidases; Cerebral-disorder;
Huntington-disease; Aggregation; Specificity; Precursor;
Release; Endopeptidase; Cell-death; Purification;
Systeme-nerveux-pathologie; Systeme-nerveux-central-
pathologie; Maladie-hereditaire; Maladie-degenerative;
Extrapyramidal-syndrome; Enzyme; Hydrolases; Peptidases;
Encephale-pathologie; Choree-Huntington; Agregation;
Specificite; Precurseur; Liberation; Endopeptidase; Mort-
cellulaire; Purification;
Sistema-nervioso-patologia; Sistema-nervosio-central-
patologia; Enfermedad-hereditaria; Enfermedad-degenerativa;
Extrapiramidal-sindrome; Enzima; Hydrolases; Peptidases;
Encefalo-patologia; Corea-Huntington; Agregacion;
Especificidad; Precursor; Liberacion; Endopeptidase; Muerte-
celular; Purificacion.
Keywords
fragments; Huntington-s-disease; inclusions; oligomer;
polyglutamine; polymer.
Subject headings
Neurology; Cell-biology-histology; Physiology-morphology;
Neurologie; Biologie-cellulaire-histologie; Physiologie-
morphologie.
Classification codes
002B17G Degenerative-and-inherited-degenerative-
diseases-of-the-nervous-system;
Leukodystrophies; Prion-diseases;
Maladies-degeneratives-et-heredodegeneratives-
du-systeme-nerveux; Leucodystrophies;
Maladies-prions;
002B17A01 Nervous-system-as-a-whole;
Systeme-nerveux-en-general.
Language
English.
Language of summary
English.
Publication type
article.
Country of publication
United Kingdom.
Publication year
2005.
Publication date
20050000.
Entry date
20070523.
Copyright statement
Copyright 2006 INIST-CNRS. All rights reserved.
Availability
Holder: INIST-CNRS, Shelf number: 4037;
Holder: Centre national de la recherche scientifique;
url: <http://cat.inist.fr/?aModele=afficheN&cpsidt=17097418>
AN Accession number 1_: 0017097418.AN.
& update 2_: 20080313.AN.
TI Title 3_: PURIFICATION WITH
HUNTINGTON.TI.
TT Title in original language 4_: PEDOPSYCHIATRIE.TT.
CF Conference information 5_: PVC SAME BRIGHTON SAME
2005.CF.
SO Source 6_: JOURNAL-OF-
NEUROCHEMISTRY.SO.
7_: J-NEUROCHEM
8_: NEUROCHEMISTRY.SO.
9_: ..ROOT SO=
10_: 0022-3042
11_: VOLUME=95
12_: ISSUE=1
13_: PAGE=125
14_: BLACKWELL.SO.
AU Authors 15_: HOFFNER-G$
16_: HOFFNER.AU.
17_: ..ROOT HOFFNER.AU.
CA Corporate author 18_: IBS ADJ RESEARCH ADJ
GROUP.CA.
IN Author affiliation 19_: PARIS.IN.
AB Abstract 20_: BRAIN ADJ REGIONS.AB.
DE Descriptors (controlled,
added by IP)(English) 21_: CELL-DEATH.DE.
(French) 22_: MORT-CELLULAIRE.DE.
(Spanish) 23_: MUERTE-CELULAR.DE.
24_: CELL ADJ DEATH.DE.
25_: ..ROOT CELL.DE.
KW Keywords (uncontrolled,
supplied by author) 26_: OLIGOMER.KW.
SH Subject headings
(English) 27_: NEUROLOGY.SH.
(French) 28_: NEUROLOGIE.SH.
29_: ..ROOT SH=
CC Classification codes 30_: 002B17G
31_: 002B#
32_: PRION-DISEASES.CC.
PA Pascal codes 33_: PRION-DISEASES.PA.
FR Francis codes 34_: 531#
35_: NORMANDY.FR.
SI Sigle codes 36_: 430#
37_: MEDICINE.SI.
EN Environment codes (none at time of writing)
PC Physics codes 38_: 425#
39_: QUANTUM-OPTICS.PC.
LG Language 40_: ENGLISH.LG.
LS Language of summary 41_: FRENCH.LS.
PT Publication type 42_: ARTICLE.PT.
43_: PT=ARTICLE
44_: ..ROOT PT=
CN Country of publication 45_: UNITED ADJ KINGDOM.CN.
YR Publication year 46_: 2005.YR.
47_: YR=2005
48_: ..ROOT YR=
DT Publication date 49_: 20051109.DT.
50_: DATE=20051109
51_: MONTH=200511
52_: YEAR=2005
ED Entry date 53_: 20070523.ED.
CP Copyright 54_: INIST.CP.
AV Availability 55_: CNRS.AV.
DO DOI (none at time of writing)
1_: SIALIC-ACID
YEAR Publication year (YYYY) 2_: ..L 1 YEAR WL 2005,2008
MONTH Publication month (YYYYMM) 3_: ..L 1 MONTH>200606
DATE Publication date (YYYYMMDD) 4_: ..L 1 DATE<20060630
HUMAN Human 5_: ..L 1 HUMAN=Y
UDATE Update date (YYYYMMDD) 6_: ..L 1 UDATE=20080726
UMONTH Update month (YYYYMM) 7_: ..L 1 UMONTH>20080530
EDATE Entry date (YYYYMMDD) 8_: ..L 1 EDATE<20080930
By paragraph - title, author _: ..P TI, AU 1-5
SHORT AN TI SO AU IN YR
MEDIUM AN TI SO AU IN AB YR
LONG AN TI SO AU IN AB DE KW SH CC YR
BIBL AN TI SO AU IN YR
FREE AN TI TT DE KW SH CC PA FR SI EN PC PT YR DT ED CP
_: ..P SHORT 1,5-8,10.
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