lipofuscinosis


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lipofuscinosis

 [lip″o-fu″sin-o´sis]
any disorder due to abnormal storage of lipofuscins.
neuronal ceroid-lipofuscinosis any of several genetic lipidoses characterized by progressive neurodegeneration, loss of vision, and a fatal course; included are janský-bielschowsky disease, vogt-spielmeyer disease, and kufs' disease. Formerly known as amaurotic familial idiocy.

lip·o·fus·ci·no·sis

(lip'ō-fyūs'i-nō'sis),
Abnormal storage of any one of a group of fatty pigments; best viewed using fluorescent microscopy.

lipofuscinosis

/lipo·fus·cin·o·sis/ (-fu″sin-o´sis) any disorder due to abnormal storage of lipofuscins.
neuronal ceroid-lipofuscinosis  any of several genetic lipidoses characterized by progressive neurodegeneration, loss of vision, and a fatal course; included are Janský-Bielschowsky disease, Vogt-Spielmeyer disease, and Kufs' disease.

lip·o·fus·ci·no·sis

(lip'ō-fyūs'i-nō'sis)
Abnormal storage of any one of a group of fatty pigments.

lip·o·fus·ci·no·sis

(lip'ō-fyūs'i-nō'sis)
Abnormal storage of any one of a group of fatty pigments.

lipofuscinosis

any disorder due to abnormal storage of lipofuscins.

ceroid lipofuscinosis
an inherited disease in sheep, cattle, dogs and cats, characterized by blindness, mental dullness and abnormal behavior. There is atrophy of the brain with extensive accumulation of lipopigments ('ceroid bodies') in nervous tissue and later viscera as well. The comparable, though possibly not identical, disease in humans is also called Batten disease.
hepatic lipofuscinosis
a deposit of lipofuscin in partly oxidized form in the livers of sheep, which gives them a deep black color and makes them unsaleable. The condition is common in areas where the mulga tree (Acacia aneura) grows and the leaves are fed to sheep as fodder.
References in periodicals archive ?
Key words: neuronal ceroid lipofuscinosis, neurodegenerative disorders, NCL, waterfowl, avian, mallard duck, Anas platyrhynchos
Diagnostic value of electron microscopy in a case of juvenile neuronal ceroid lipofuscinosis.
StemCells says the proposed Phase I trial is designed to evaluate the safety and preliminary efficacy of HuCNS-SC as a treatment of infantile and late-infantile neuronal ceroid lipofuscinosis.
The gene defect causes the extremely rare disease late-infantile neuronal ceroid lipofuscinosis that is fatal by 8 years of age.
The proposed Phase 1 trial is designed to evaluate the safety and preliminary efficacy of HuCNS-SC for the treatment of infantile and late-infantile neuronal ceroid lipofuscinosis (NCL), the most severe forms of a group of disorders commonly referred to as Batten disease.
Characterization of endopeptidase activity of tripeptidyl peptidase-I/CLN2 protein which is deficient in classical late infantile neuronal ceroid lipofuscinosis.
StemCells has completed a Phase I clinical trial of its HuCNS-SC cells for the treatment of a Neuronal Ceroid Lipofuscinosis (NCL), a fatal brain disorder in children.
The proposed Phase I trial is designed to investigate the safety of HuCNS-SC in the treatment of infantile and late-infantile neuronal ceroid lipofuscinosis (NCL), the most severe forms of a group of disorders commonly referred to as Batten disease.
Mutations in the palmitoyl protein thicesterase gene causing infantile neuronal ceroid lipofuscinosis.
NASDAQ: STEM) today provided an update on the ongoing clinical development program of its proprietary HuCNS-SC([R]) product candidate (purified human neural stem cells) for neuronal ceroid lipofuscinosis (NCL), often referred to as Batten disease.
Late Infantile Neuronal Ceroid Lipofuscinosis (LINCL) is an autosomal recessive genetic disorder that causes degeneration of the central nervous system.