lipofuscin

(redirected from Ceroid)
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lipofuscin

[‚lip·ə′fyüs·ən]
(biochemistry)
Any of a group of lipid pigments found in cardiac and smooth muscle cells, in macrophages, and in parenchyma and interstitial cells; differential reactions include sudanophilia, Nile blue staining, fatty acid, glycol, and ethylene.
References in periodicals archive ?
A fatal lysosomal storage disease of the nervous system caused by autosomal-recessive mutations in the CLN1 gene, also known as infantile neuronal ceroid lipofuscinosis, CLN1 disease is an inherited genetic disease that primarily affects the nervous system in newborns and progresses rapidly.
M2 PHARMA-March 2, 2018-EMA Grants Polaryx Therapeutics Orphan Drug Designation for PLX-200 for Treatment of Neuronal Ceroid Lipofuscinosis
A fatal lysosomal storage disease of the nervous system caused by autosomal-recessive mutations in the CLN1 gene, also known as infantile neuronal ceroid lipofuscinosis (INCL), infantile Batten disease is an inherited fatal genetic disease that primarily affects the nervous system in newborns and progresses rapidly.
About Infantile Batten Disease: Infantile neuronal ceroid lipofuscinosis (INCL) is a severe lysosomal disease caused by mutations in the CLN1 gene, which encodes the soluble lysosomal enzyme Palmitoyl-Protein-Thioesterase-1 (PPT1) and result in osmiophilic granules accumulating in lysosomes and leading to neuroinflammation, neurodegeneration and death.
In April 2017, the company received the second voucher following approval of Brineura, a new biological product for patients with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), also known as tripeptidyl peptidase 1 (TPP1) deficiency, a form of Batten disease.
Batten disease is commonly used to make reference to all forms of  Neuronal Ceroid Lipofuscinoses (NCL).
Originally described by Hermansky and Pudlak in 1959, (77) Hermansky-Pudlak Syndrome (HPS) is caused by a related group of autosomal recessive disorders that are characterized by the triad of oculocutaneous albinism, bleeding diathesis due to a platelet storage pool deficiency, and lysosomal accumulation of ceroid lipofuscin.
Brineura is the first FDA-approved treatment to slow loss of walking ability in symptomatic pediatric patients 3 years of age and older with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), also known as tripeptidyl peptidase-1 (TPP1) deficiency.
Serum antibodies to oxidized, low-density lipoprotein and ceroid in chronic periaortitis.
The cortex is involved in rare disorders like neuronal ceroid lipofuscinoses and Rett syndrome.
It forms large aggregates and increases in size after active phagocytosis of heterogeneous materials such as detritus, melanin (brown or black color), ceroid or hemosiderin and lipofuscin (natural yellowish color or blue) (AGIUS, 1980; MUNSHI & DUTTA, 1996).
Certain tissue pathologies and tissue components were also quantified by direct counts, including cases of hemocytic infiltration that were scored separately as focal and diffuse (Kim & Powell 2004), granulocytomas (Lowe & Moore 1979), and ceroid bodies (Mackin 1951, Stein & Mackin 1955; also termed brown cells by Zaroogian et al.