Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1864663 (HCC)
 2,985 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The structure and distribution of hyaline-cell cartilage (chondroid) (HCC) in the heads of teleosts has been studied in 48 species from 16 families. The tissue is pale-staining and has closely-packed, hyaline cells that are separated by a small quantity of matrix. The matrix has only a mild affinity for alcian blue and the cells are not shrunken within lacunae. Two subtypes of the tissue are here described--fibrohyaline-cell cartilage (chondroid) where collagen fibres are prominent in the matrix, and lipohyaline-cell cartilage where fat and hyaline cells are intermingled. An elastic hyaline-cell cartilage has been described previously. Associations of HCC with dense fibrous connective tissue, mucochondroid, hyaline cartilage and bone are described. Lists are provided of membrane and cartilages bones to which the tissue is attached and of species in which it is common. Suitable 'type examples' for reference and for further study include the cartilage in the rostral folds of the red-tailed black shark, Labeo bicolor and the flying fox, Epalzeorhynchus kalopterus. HCC occurs in lips and rostral folds, in pre-palatine and submaxillary menisci, in ligaments, at the anterior end of the basihyal, in the pectoral girdle, in adhesive discs, in gill arches, beneath the basioccipital chewing pad, in barbels, next to the facial nerve, around the olfactory region and in the core of the nasal skin flaps. It is a particularly important tissue in cyprinids and related fish, and enormous masses of it are present in the black shark, Morulius chrysophekadion and the Hong Kong pleco, Pseudogastromyzon myersi. It acts as a damper against the contractions of the heart or the pressure of occluding pharyngeal teeth, and it provides the mouth region of bottom-dwelling, algal eaters with flexible support. In relation to Schaffer's classification of supporting tissues, I confirm a distinction between HCC and Zellknorpel.
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PMID:Hyaline-cell cartilage (chondroid) in the heads of teleosts. 291 51

"Hypomyelination and Congenital Cataract", HCC (MIM #610532), is an autosomal recessive disorder characterized by congenital cataract and diffuse cerebral and peripheral hypomyelination. HCC is caused by deficiency of Hyccin, a protein whose biological role has not been clarified yet. Since the identification of the cell types expressing a protein of unknown function can contribute to define the physiological context in which the molecule is explicating its function, we analyzed the pattern of Hyccin expression in the central and peripheral nervous system (CNS and PNS). Using heterozygous mice expressing the b-galactosidase (LacZ) gene under control of the Hyccin gene regulatory elements, we show that the gene is primarily expressed in neuronal cells. Indeed, Hyccin-LacZ signal was identified in CA1 hippocampal pyramidal neurons, olfactory bulb, and cortical pyramidal neurons, while it did not colocalize with oligodendroglial or astrocytic markers. In the PNS, Hyccin was detectable only in axons isolated from newborn mice. In the brain, Hyccin transcript levels were higher in early postnatal development (postnatal days 2 and 10) and then declined in adult mice. In a model of active myelinogenesis, organotypic cultures of rat Schwann cells (SC)/Dorsal Root Ganglion (DRG) sensory neurons, Hyccin was detected along the neurites, while it was absent from SC. Intriguingly, the abundance of the molecule was upregulated at postnatal days 10 and 15, in the initial steps of myelinogenesis and then declined at 30 days when the process is complete. As Hyccin is primarily expressed in neurons and its mutation leads to hypomyelination in human patients, we suggest that the protein is involved in neuron-to-glia signalling to initiate or maintain myelination.
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PMID:Hyccin, the molecule mutated in the leukodystrophy hypomyelination and congenital cataract (HCC), is a neuronal protein. 2246 84