Biomaterial Database

Ceroid, lipofuscin and the ceroid-lipofuscinoses (Batten disease). 1993

R D Jolly, and R R Dalefield, and D N Palmer

Department of Veterinary Pathology and Public Health, Massey University, Palmerston North, New Zealand.

UI	MeSH Term	Description	Entries
D008062	Lipofuscin	A naturally occurring lipid pigment with histochemical characteristics similar to ceroid. It accumulates in various normal tissues and apparently increases in quantity with age.
D009336	Necrosis	The death of cells in an organ or tissue due to disease, injury or failure of the blood supply.
D009472	Neuronal Ceroid-Lipofuscinoses	A group of severe neurodegenerative diseases characterized by intracellular accumulation of autofluorescent wax-like lipid materials (CEROID; LIPOFUSCIN) in neurons. There are several subtypes based on mutations of the various genes, time of disease onset, and severity of the neurological defects such as progressive DEMENTIA; SEIZURES; and visual failure.	Batten Disease,Ceroid Lipofuscinosis, Neuronal, 4B, Autosomal Dominant,Ceroid-Lipofuscinosis, Neuronal,Jansky-Bielschowsky Disease,Kufs Disease,Santavuori-Haltia Disease,Spielmeyer-Vogt Disease,Adult Neuronal Ceroid Lipofuscinosis,Amaurotic Idiocy, Adult Type,Batten-Mayou Disease,Batten-Spielmeyer-Vogt Disease,CLN3-Related Neuronal Ceroid-Lipofuscinosis,CLN4A,CLN4B,Ceroid Lipofuscinosis, Neuronal 3, Juvenile,Ceroid Lipofuscinosis, Neuronal 4,Ceroid Lipofuscinosis, Neuronal, 3,Ceroid Lipofuscinosis, Neuronal, 4A, Autosomal Recessive,Ceroid Lipofuscinosis, Neuronal, Parry Type,Ceroid Storage Disease,Infantile Neuronal Ceroid Lipofuscinosis,Juvenile Batten Disease,Juvenile Cerebroretinal Degeneration,Juvenile Neuronal Ceroid Lipofuscinosis,Kuf's Disease,Kufs Disease Autosomal Recessive,Kufs Disease, Autosomal Dominant,Kufs Disease, Autosomal Recessive,Kufs Type Neuronal Ceroid Lipofuscinosis,Late-Infantile Neuronal Ceroid Lipofuscinosis,Lipofuscin Storage Disease,Lipofuscinosis, Neuronal Ceroid,Neuronal Ceroid Lipofuscinosis,Neuronal Ceroid Lipofuscinosis Juvenile Type,Neuronal Ceroid Lipofuscinosis, Adult,Neuronal Ceroid Lipofuscinosis, Adult Type,Neuronal Ceroid Lipofuscinosis, Infantile,Neuronal Ceroid Lipofuscinosis, Juvenile,Neuronal Ceroid Lipofuscinosis, Late Infantile,Neuronal Ceroid Lipofuscinosis, Late-Infantile,Neuronal Ceroid-Lipofuscinosis,Spielmeyer-Sjogren Disease,Vogt Spielmeyer Disease,Vogt-Spielmeyer Disease,Batten Disease, Juvenile,Batten Diseases, Juvenile,Batten Mayou Disease,Batten Spielmeyer Vogt Disease,CLN3 Related Neuronal Ceroid Lipofuscinosis,CLN3-Related Neuronal Ceroid-Lipofuscinoses,CLN4As,Cerebroretinal Degeneration, Juvenile,Cerebroretinal Degenerations, Juvenile,Ceroid Lipofuscinosis, Neuronal,Ceroid Storage Diseases,Ceroid-Lipofuscinosis, CLN3-Related Neuronal,Disease, Ceroid Storage,Disease, Juvenile Batten,Disease, Kuf's,Disease, Lipofuscin Storage,Disease, Spielmeyer-Sjogren,Disease, Vogt Spielmeyer,Disease, Vogt-Spielmeyer,Jansky Bielschowsky Disease,Juvenile Batten Diseases,Juvenile Cerebroretinal Degenerations,Kuf Disease,Lipofuscin Storage Diseases,Neuronal Ceroid Lipofuscinoses,Neuronal Ceroid-Lipofuscinoses, CLN3-Related,Neuronal Ceroid-Lipofuscinosis, CLN3-Related,Santavuori Haltia Disease,Spielmeyer Disease, Vogt,Spielmeyer Sjogren Disease,Spielmeyer Vogt Disease,Storage Disease, Ceroid,Storage Disease, Lipofuscin
D009474	Neurons	The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the NERVOUS SYSTEM.	Nerve Cells,Cell, Nerve,Cells, Nerve,Nerve Cell,Neuron
D010786	Photoreceptor Cells	Specialized cells that detect and transduce light. They are classified into two types based on their light reception structure, the ciliary photoreceptors and the rhabdomeric photoreceptors with MICROVILLI. Ciliary photoreceptor cells use OPSINS that activate a PHOSPHODIESTERASE phosphodiesterase cascade. Rhabdomeric photoreceptor cells use opsins that activate a PHOSPHOLIPASE C cascade.	Ciliary Photoreceptor Cells,Ciliary Photoreceptors,Rhabdomeric Photoreceptor Cells,Rhabdomeric Photoreceptors,Cell, Ciliary Photoreceptor,Cell, Photoreceptor,Cell, Rhabdomeric Photoreceptor,Cells, Ciliary Photoreceptor,Cells, Photoreceptor,Cells, Rhabdomeric Photoreceptor,Ciliary Photoreceptor,Ciliary Photoreceptor Cell,Photoreceptor Cell,Photoreceptor Cell, Ciliary,Photoreceptor Cell, Rhabdomeric,Photoreceptor Cells, Ciliary,Photoreceptor Cells, Rhabdomeric,Photoreceptor, Ciliary,Photoreceptor, Rhabdomeric,Photoreceptors, Ciliary,Photoreceptors, Rhabdomeric,Rhabdomeric Photoreceptor,Rhabdomeric Photoreceptor Cell
D002566	Ceroid	A naturally occurring lipid pigment with histochemical characteristics similar to lipofuscin. It accumulates in various tissues in certain experimental and pathological conditions.
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man