Ovine ceroid-lipofuscinosis
Ovine ceroid lipofuscinosis : a thesis presented in partial fulfilment (70%) of the requirements for the degree of Master of Veterinary Science at Massey University
... Granular electron dense inclusion in a macrophage in the rectal wall of a 5 months old normal lamb. These inclusions are encountered in both normal and affected sheep, and carry no significance in the diagnosis of ...
15
Neuropathology of ovine ceroid lipofuscinosis : a thesis presented in partial fulfilment (30%) of the requirements for the degree of Master of Philosophy in Veterinary Pathology at Massey University
... There are a number o f pos sible mechanisms that could account for a lysosoma l proteinosis , such as defi ciency of lysosomal protea s e or its control , secondary perturbation of lysosomal function similar to that ...
141
Ovine ceroid lipofuscinosis : a thesis presented in partial fulfilment (70%) of the requirements for the degree of Master of Veterinary Science at Massey University
... Gonatas et al. ( 1968 ) , and Duf fy et al. ( 19 6 8 ) attempted to correla te ultrastructural findings wi th c l in i c al ly d i f fe rentiable entities o f the neuronal ceroid - l ipo fus cinoses . However , ...
165
Biochemical studies on ovine ceroid lipofuscinosis : a thesis presented in partial fulfilment of the requirements for the degree of Doctor of Philosophy in Veterinary Pathology at Massey University
... Permission is given for a copy to be downloaded by an individual for the purpose of research and private study only.. The thesis may not be reproduced elsewhere without the permission of[r] ...
115
The retinal pathology of ovine ceroid lipofuscinosis : a thesis presented in fulfilment of the requirement for the degree of Master of Veterinary Science at Massey University
... In 1978 skin biopsy examination led to the diagnosis of ceroid lipofuscinosis in four of the 51 lambs born that year and only those four animals developed the disease. Skin biopsies from the lambs born in 1979 ...
106
Neuropathology of ovine ceroid lipofuscinosis : a thesis presented in partial fulfilment (30%) of the requirements for the degree of Master of Philosophy in Veterinary Pathology at Massey University
... I also wish to acknowledge the help of Mr. M.J. B i rtles of the Department of Physi ol ogy and Anatomy in the preparati on of t issues for the immuno cytochemi cal study , Dr. A.S. Davies of the Department of Physi ol ...
15
Acid ceramidase deficiency: Farber disease and SMA-PME
... AAV9: Adeno-associated virus serotype 9; ACDase: Acid ceramidase; CHO: Chinese hamster ovary; CLN2: Neuronal ceroid lipofuscinosis type 2; CRP: C- reactive protein; CNS: Central nervous [r] ...
19
Longitudinal in vivo monitoring of the neuropathology in ovine neuronal ceroid lipofuscinoses
... The main findings of this thesis together with previous descriptions of the clinical development, in vivo monitoring and neuropathological cascades from the other two models are summarised in Table 8.1, Table 8.2 and ...
146
The molecular genetics of Turkish variant late infantile neuronal ceroid lipofuscinosis (LINCL)
... neuronal ceroid lipofuscinoses (NCL: also known as Batten disease) are a group o f childhood neurodegenerative ...f ceroid-lipofuscin- like lipopigments (Zeman and Dyken 1969) which differed from Tay-Sach ...
266
Glial cells are functionally impaired in juvenile neuronal ceroid lipofuscinosis and detrimental to neurons
... The neuronal ceroid lipofuscinoses (NCLs or Batten disease) are a group of inherited, fatal neurodegenerative disorders of childhood. In these disorders, glial (microglial and astrocyte) activation typically ...
21
Global and Widespread Local White Matter Abnormalities in Juvenile Neuronal Ceroid Lipofuscinosis
... juvenile neuronal ceroid lipofuscinosis visualized on axial (A), coronal (B), and sagittal (C) slices. The white matter tract skeleton is visualized in green, and the significant results are visualized from ...
6
Using the social amoeba Dictyostelium to study the functions of proteins linked to neuronal ceroid lipofuscinosis
... neurological disorder that affects both children and adults [16]. The disease, which is the most common form of neu- rodegeneration in children, affects all ethnicities world- wide. The highest rates of incidence are ...
15
Cysteine string protein (CSP) and its role in preventing neurodegeneration
... neuronal ceroid lipofuscinoses, which is a large genetically heterogeneous class of neurodegenerative disorders defined by these two essential features [86, ...
32
Ophthalmic examination as a means to diagnose Subacute Sclerosing Panencephalitis: an optical coherence tomography and ultrawide field imaging evaluation
... MRI: Magnetic resonance imaging; NCL: Neuronal ceroid lipofuscinosis; OCT: Optical coherence tomography; RE: Right eye; SSPE: Subacute sclerosing panencephalitis; UWFA: Ultrawide field f[r] ...
5
MR imaging and localized proton MR spectroscopy in late infantile neuronal ceroid lipofuscinosis
... Neuronal ceroid lipofuscinosis (NCL) refers to a heterogeneous group of inherited neurodegenerative disorders characterized by the accumulation of lipo- pigment within the lysosomes of neurons and other ...
5
Chronic oral administration of minocycline to sheep with ovine CLN6 neuronal ceroid lipofuscinosis maintains pharmacological concentrations in the brain but does not suppress neuroinflammation or disease progression
... neuronal ceroid lipofuscinoses (NCLs; or Batten disease) are a group of fatal inherited human neurode- generative diseases affecting about 1:12,500 live births worldwide ...
9
Selective depletion of microglial progranulin in mice is not sufficient to cause neuronal ceroid lipofuscinosis or neuroinflammation
... neuronal ceroid lipofuscinosis (NCL) in the rare case of homozygous-null mutations ...and lipofuscinosis in the thalamus, which spreads to other brain regions and is ultimately followed by extensive ...
11
Compromised astrocyte function and survival negatively impact neurons in infantile neuronal ceroid lipofuscinosis
... The neuronal ceroid lipofuscinoses (NCLs) are the most common cause of childhood dementia and are invariably fatal. Early localized glial activation occurs in these disorders, and accurately predicts where ...
21
A novel cathepsin D mutation in 2 siblings with late infantile neuronal ceroid lipofuscinosis
... Neuronal ceroid-lipofuscinosis (NCL) is a heterogeneous and rare lysosomal storage disorder characterized by the accumulation of autofluorescent materials—ceroid and lipofuscin—in the ...
5
Using to fight human neurodegenerative diseases.
... elegans null mutant model of the rare autosomal dominant human ND known as adult-onset neuronal ceroid lipofuscinosis ANCL, but also ameliorated the mobility defect and short lifespan of[r] ...
334