Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Autofluorescence emission spectra of neuronal lipopigment in mucopolysaccharidosis (Sanfilippo's syndrome).
Acta Neuropathol
May 1989
Department of Psychiatry, University of Cambridge Clinical School, Addenbrooke's Hospital, Great Britain.
Characteristics of intraneuronal lipopigment in two siblings with Sanfilippo's syndrome are reported. A lipopigment emission spectrum probably reflects its composition and the (uncorrected) autofluorescence emission spectra results are compared with spectra from non-diseased tissue and from previously reported childhood-onset neuronal ceroidlipofuscinoses (ceroidoses), adult-onset ceroidosis (Kufs' disease) and animal ceroidoses. Values derived from the emission spectra from Sanfilippo's syndrome could be distinguished from those obtained from equivalent regions of non-diseased brains and were within the range of abnormal values previously reported from accumulations of pigment in various types of neuronal ceroidosis.
View Article and Find Full Text PDF4-Hydroxynonenal: a specific indicator for canine neuronal-retinal ceroidosis.
Am J Med Genet Suppl
March 1989
Department of Pathology, Indiana University School of Medicine, Indianapolis 46223.
Previous attempts to demonstrate abnormalities in lipid peroxidation in various forms of the neuronal ceroid-lipofuscinoses (NCL) have been unrewarding up to and including the peroxide level (peroxidase). In this experiment a survey was made in a canine model of NCL to study the relative concentration of 4-hydroxynonenal (HNE), a fragment derived from an acute oxidation product of unsaturated fatty acids. Peripheral blood cells and various tissues from an affected and a normal control dog were surveyed.
View Article and Find Full Text PDFAnimal model: ceroidosis (ceroid-lipofuscinosis) in Australian cattle dogs.
Am J Med Genet
April 1987
Ceroid-lipofuscinosis is described in Australian Cattle dogs. Lesions included storage of ceroid-lipofuscin in most tissues with characteristic ultrastructural inclusion body patterns in neurons and other cells. Dolichol concentration of the affected dog's brain was similar to those in age-matched control dog brains.
View Article and Find Full Text PDFNeuronal ceroidosis was observed in an 18-month-old male Blue Heeler dog which was euthanized after showing a progressive gait and behavior abnormality, depression, paresthesia, and vision deterioration. The brain was slightly atrophic. Histopathology revealed autofluorescent, periodic acid-Schiff, luxol fast blue, and oil red O-positive intracytoplasmic granules in the neurons of the brain and spinal cord.
View Article and Find Full Text PDFThe study reports characteristics of the autofluorescence emission spectra from abnormal accumulations of intraneuronal lipopigment in a case of adult-onset neuronal ceroidosis (Kufs' disease). These are compared with emission spectra from 14 non-diseased brains, and from cases of childhood-onset neuronal ceroidoses (Batten's disease) and animal ceroidoses which have been previously reported. The emission spectra derived from the case of Kufs' disease could be distinguished from the spectra from the non-diseased brains and from the cases of Batten's disease and animal ceroidoses.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!