X-ray microprobe analysis of the retina and RPE in sheep with ovine ceroid-lipofuscinosis
Journal Article
·
· Neurobiology of Aging; (USA)
- Univ. of Florida, Gainesville (USA)
Ovine ceroid-lipofuscinosis (OCL) is one animal model for the human condition, and because autofluorescent lipopigments are prominent in the brain and eye, it may also prove useful as a model for aging. For example, a progressive decline in electrical recording from brain and retina are observed in both aging and OCL. Samples of retinal and retinal pigment epithelial (RPE) tissues were obtained from a young control. 2 animals with OCL and a normal aged sheep. Specimens were cryo-fractured and examined by scanning electron microscopy/x-ray microanalysis. Measurements made of 6 individual cells in the ganglion layer of OCL specimens, the remainder of the retina, and RPE showed age-related changes in zinc, iron, and copper which were associated with lipopigment accumulation in the RPE. There was marked decrease in phosphate, sulfur, and manganese levels, as photoreceptor cells and their outer segments are lost in the disease process. This is the first report of metal analysis in the retina and RPE in a disease entity, and as a function of normal aging.
- OSTI ID:
- 6008445
- Journal Information:
- Neurobiology of Aging; (USA), Journal Name: Neurobiology of Aging; (USA) Vol. 11:6; ISSN 0197-4580; ISSN NEAGD
- Country of Publication:
- United States
- Language:
- English
Similar Records
Analysis of the RPE sheet in the rd10 retinal degeneration model
CLRN1 Is Nonessential in the Mouse Retina but Is Required for Cochlear Hair Cell Development
Genetic heterogeneity in neuronal ceroid lipofuscinosis (NCL): Evidence that the late-infantile subtype (Jansky-Bielschowsky disease; CLN2) is not an allelic form of the juvenile or infantile subtypes
Conference
·
Mon Jan 03 23:00:00 EST 2011
·
OSTI ID:1045415
CLRN1 Is Nonessential in the Mouse Retina but Is Required for Cochlear Hair Cell Development
Journal Article
·
Fri Aug 14 00:00:00 EDT 2009
· PLoS Genetics
·
OSTI ID:1627277
Genetic heterogeneity in neuronal ceroid lipofuscinosis (NCL): Evidence that the late-infantile subtype (Jansky-Bielschowsky disease; CLN2) is not an allelic form of the juvenile or infantile subtypes
Journal Article
·
Fri Oct 01 00:00:00 EDT 1993
· American Journal of Human Genetics; (United States)
·
OSTI ID:5136015
Related Subjects
550602* -- Medicine-- External Radiation in Diagnostics-- (1980-)
62 RADIOLOGY AND NUCLEAR MEDICINE
AGE DEPENDENCE
ANIMAL TISSUES
ANIMALS
BODY
BODY AREAS
BRAIN
CENTRAL NERVOUS SYSTEM
CHEMICAL ANALYSIS
CHEMICAL COMPOSITION
DISEASES
DOMESTIC ANIMALS
ELECTROMAGNETIC RADIATION
ELECTRON MICROPROBE ANALYSIS
EPITHELIUM
EYES
FACE
HEAD
IONIZING RADIATIONS
MAMMALS
MICROANALYSIS
NERVOUS SYSTEM
ORGANS
RADIATIONS
RETINA
RUMINANTS
SENSE ORGANS
SENSE ORGANS DISEASES
SHEEP
TISSUES
VERTEBRATES
X RADIATION
62 RADIOLOGY AND NUCLEAR MEDICINE
AGE DEPENDENCE
ANIMAL TISSUES
ANIMALS
BODY
BODY AREAS
BRAIN
CENTRAL NERVOUS SYSTEM
CHEMICAL ANALYSIS
CHEMICAL COMPOSITION
DISEASES
DOMESTIC ANIMALS
ELECTROMAGNETIC RADIATION
ELECTRON MICROPROBE ANALYSIS
EPITHELIUM
EYES
FACE
HEAD
IONIZING RADIATIONS
MAMMALS
MICROANALYSIS
NERVOUS SYSTEM
ORGANS
RADIATIONS
RETINA
RUMINANTS
SENSE ORGANS
SENSE ORGANS DISEASES
SHEEP
TISSUES
VERTEBRATES
X RADIATION