Alterations of striatal neurons in benign hereditary chorea.
Benign hereditary chorea (BHC) recently has been associated with mutations in TITF-1 gene, although a pathological study of an individual with BHC and a TITF-1 mutation revealed no significant gross or microscopic abnormalities using standard methods. Immunohistochemical staining of striatal tissue from a BHC-affected postmortem brain was performed using antibodies against neurotransmitters of interneurons whose tangential migration is mediated by TITF-1. There was a loss of most TITF-1-mediated striatal interneurons in the BHC specimen compared to four matched control brains.
Parkinson's Disease Research, Education and Clinical Center (PADRECC), Philadelphia VA Hospital, Philadelphia, Pennsylvania 19104, USA. galit.kleiner-fisman@med.va.gov
Galit
G
Kleiner-Fisman
Noel Y
NY
Calingasan
Mary
M
Putt
June
J
Chen
M Flint
MF
Beal
Anthony E
AE
Lang
D000906:Antibodies / Q000276:immunology; D002465:Cell Movement; D002819:Chorea / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D002883:Chromosomes, Human, Pair 14 / Q000235:genetics; D003342:Corpus Striatum / Q000378:metabolism / Q000473:pathology*; D017809:Fatal Outcome; D006801:Humans; D007150:Immunohistochemistry; D007395:Interneurons / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D008875:Middle Aged; D009474:Neurons / Q000378:metabolism / Q000473:pathology*; D018377:Neurotransmitter Agents / Q000276:immunology; D009687:Nuclear Proteins / Q000235:genetics / Q000378:metabolism; D017354:Point Mutation / Q000235:genetics; D000074482:Thyroid Nuclear Factor 1; D014157:Transcription Factors / Q000235:genetics / Q000378:metabolism
D000906:Antibodies; D018377:Neurotransmitter Agents; D009687:Nuclear Proteins; D000074482:Thyroid Nuclear Factor 1; D014157:Transcription Factors