Concurrence of Danish dementia and cataract: insights from the interactions of dementia associated peptides with eye lens alpha-crystallin.
Familial Danish Dementia (FDD) is an autosomal disease, which is distinguished by gradual loss of vision, deafness, progressive ataxia and dementia. Cataract is the first manifestation of the disease. In this article, we demonstrate a specific correlation between the poisoning of the chaperone activity of the rat eye lens alpha-crystallins, loss of lens transparency in organ culture by the pathogenic form of the Danish dementia peptide, i.e. the reduced Danish dementia peptide (redADan peptide), by a combination of ex vivo, in vitro, biophysical and biochemical techniques. The interaction of redADan peptide and lens crystallins are very specific when compared with another chaperone, HSP-70, underscoring the specificity of the pathogenic form of Danish dementia peptide, redADan, for the early onset of cataract in this disease.
Surolia
Ira
I
Department of Biochemistry, South Campus, Delhi University, New Delhi, India. surolia@nii.res.in
Sinha
Sharmistha
S
Sarkar
Debi Prasad
DP
Reddy
P Yadagiri
PY
Reddy
G Bhanuprakash
GB
Surolia
Avadhesha
A
D048868:Adaptor Proteins, Signal Transducing; D000375:Aging; D016229:Amyloid beta-Peptides / Q000633:toxicity; D000818:Animals; D001259:Ataxia / Q000150:complications; D002386:Cataract / Q000150:complications*; D003704:Dementia / Q000150:complications*; D004195:Disease Models, Animal; D006801:Humans; D007908:Lens, Crystalline / Q000473:pathology*; D008562:Membrane Glycoproteins; D008565:Membrane Proteins / Q000633:toxicity; D018832:Molecular Chaperones / Q000502:physiology; D019636:Neurodegenerative Diseases / Q000453:epidemiology; D051381:Rats; D014786:Vision Disorders / Q000150:complications; D038201:alpha-Crystallins / Q000502:physiology*
D048868:Adaptor Proteins, Signal Transducing; D016229:Amyloid beta-Peptides; C120083:ITM2B protein, human; D008562:Membrane Glycoproteins; D008565:Membrane Proteins; D018832:Molecular Chaperones; D038201:alpha-Crystallins