High expression on Kunitz-type protease inhibitor-containing substances in the cerebral vessels of patients with Down syndrome.
Down syndrome (DS) brains, from 19 gestational weeks to 50 years of age were studied by immunohistochemical methods with a polyclonal antibody against synthetic peptide comprising part of the Kunitz-type protease inhibitor (KPI) domain of Alzheimer disease amyloid precursor protein (APP), residues 301 to 323 of APP 770. In DS, positive KPI immunoreactivity was observed in early infancy and from child to adulthood on the tunica media of the arteries in the leptomeninges, cerebral cortex and white matter, but negative or little in controls. In DS with Alzheimer type dementia, KPI immunoreactivity in the arteries was reduced, but a gross granular reactivity was noted in neurons and glial cells. The high expression of KPI in DS vessels may be one of the predisposing factors to vascular diseases and amyloid deposition associated with DS.
Suzuki
A
A
Department of Mental Retardation and Birth Defect Research, National Center of Neurology and Psychiatry, Tokyo.
Takashima
S
S
Mizuguchi
M
M
Kato
M
M
Kunishita
T
T
Tabira
T
T
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000544:Alzheimer Disease / Q000209:etiology / Q000378:metabolism*; D016564:Amyloid beta-Protein Precursor / Q000276:immunology*; D000818:Animals; D002536:Cerebral Arteries / Q000737:chemistry* / Q000196:embryology; D002550:Cerebral Veins / Q000737:chemistry / Q000196:embryology; D002648:Child; D002675:Child, Preschool; D004314:Down Syndrome / Q000150:complications / Q000378:metabolism*; D005260:Female; D005625:Frontal Lobe / Q000098:blood supply; D005865:Gestational Age; D006801:Humans; D007124:Immunoenzyme Techniques; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008578:Meninges / Q000098:blood supply; D008875:Middle Aged; D009124:Muscle Proteins / Q000032:analysis; D009131:Muscle, Smooth, Vascular / Q000737:chemistry; D010446:Peptide Fragments / Q000276:immunology*; D011817:Rabbits
D016564:Amyloid beta-Protein Precursor; D009124:Muscle Proteins; D010446:Peptide Fragments; C093596:amyloid beta-protein precursor 770 (301-323)