From the Medical Literature - November 1998

Record  1

  Cognitive functioning in adults with Williams syndrome.
  Howlin P; Davies M; Udwin O
  Department  of  Psychology,  St George's Hospital Medical School, London,
U.K.
  J  Child  Psychol  Psychiatry (ENGLAND)   Feb 1998,  39 (2) p183-9,  ISSN
0021-9630 
  Languages: ENGLISH
  Document type: JOURNAL ARTICLE

  This  paper  reports  the  results of cognitive, linguistic, and academic
assessments in a representative sample of 62 adults with Williams syndrome.
The  average age of the group was 26 years and their mean full scale IQ was
61.  Differences  between  Verbal and Performance IQ, and between receptive
and  expressive  language  skills,  were  smaller  than  generally found in
studies of children with this condition. However, an examination of subtest
scores  revealed  an  almost  identical  cognitive profile to that found in
children. Skills in other areas, such as reading, spelling, arithmetic, and
social adaptation remained at a low level, with functioning generally being
around   a   6-8-year   age  equivalent.  The  consistency  of  reports  on
intellectual  abilities in both child and adult studies of individuals with
Williams  syndrome  lends  increased  support  to  the notion of a syndrome
specific pattern of cognitive, linguistic, and adaptive functioning.

Record  2

  Transmembrane  neuregulins  interact  with  LIM  kinase  1, a cytoplasmic
protein kinase implicated in development of visuospatial cognition.
  Wang JY; Frenzel KE; Wen D; Falls DL
  Department of Biology, Emory University, Atlanta, Georgia 30322, USA.
  J  Biol  Chem (UNITED  STATES)   Aug  7  1998,  273 (32) p20525-34,  ISSN
0021-9258   
  Languages: ENGLISH
  Document type: JOURNAL ARTICLE

  The neuregulins are receptor tyrosine kinase ligands that play a critical
role  in  the  development of the heart, nervous system, and breast. Unlike
many  extracellular  signaling  molecules,  such as the neurotrophins, most
neuregulins  are  synthesized  as  transmembrane proteins. To determine the
functions  of  the  highly  conserved  neuregulin cytoplasmic tail, a yeast
two-hybrid screen was performed to identify proteins that interact with the
157-amino   acid   sequence   common   to  the  cytoplasmic  tails  of  all
transmembrane neuregulin isoforms. This screen revealed that the neuregulin
cytoplasmic  tail  interacts  with the LIM domain region of the nonreceptor
protein  kinase  LIM  kinase  1 (LIMK1). Interaction between the neuregulin
cytoplasmic tail and full-length LIMK1 was demonstrated by in vitro binding
and  co-immunoprecipitation  assays. Transmembrane neuregulins with each of
the three known neuregulin cytoplasmic tail isoforms interacted with LIMK1.
In contrast, the cytoplasmic tail of TGF-alpha did not interact with LIMK1.
In  vivo,  neuregulin  and  LIMK1  are  co-localized  at  the neuromuscular
synapse, suggesting that LIMK1, like neuregulin, may play a role in synapse
formation  and maintenance. To our knowledge, LIMK1 is the first identified
protein  shown to interact with the cytoplasmic tail of a receptor tyrosine
kinase ligand.

Record  3
  Persistent left superior vena cava [letter]
  Del Campo C
  Tex Heart Inst J (UNITED STATES)   1998,  25 (2) p161,  ISSN 0730-2347
Journal Code: BLN
  Languages: ENGLISH
  Document type: LETTER