Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029713 (immaturity)
 4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The interpretation of rectal suction mucosal biopsies taken for the purpose of ruling out Hirschsprung's disease (HD) can be especially difficult in neonatal patients because of ganglion cell (GC) immaturity. Acetylcholinesterase histochemistry on frozen sections can be helpful but requires experience and may be complicated by excessive mucosal hemorrhage. The authors retrospectively have studied 27 patients, including 11 patients with HD, on whom conventionally fixed and embedded tissue was available, using an immunoperoxidase system directed against neuron-specific enolase (NSE) and S100 protein. NSE immunostaining produced intense staining of GC perikarya, greatly facilitating recognition of small immature forms. S100 immunostaining also highlighted GC as prominent negative stained cells surrounded by the positivity of Schwann cells. Both stains were helpful in demonstrating the overall pattern of microinnervation and its relationship to possible GC. The authors conclude that NSE and S100 immunostaining may facilitate interpretation of rectal mucosal biopsies when Hirschsprung's disease is being considered as a possibility.
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PMID:Immunohistochemistry as an aid in the diagnosis of Hirschsprung's disease. 257 28

The benign or malignant nature and the biological behaviour of immature teratomas of childhood are difficult to predict. The age of the patient at diagnosis, the anatomical site of the tumour and the degree of immaturity are considered to be important prognostic parameters. In this study the pathological-anatomical findings and the most important clinical features of 21 patients with immature teratoma (including two with supposedly malignant monodermal teratoma with immature neuroectodermal structures) were evaluated. Significant results were: Twelve tumours occurred in infancy or infants who died in the perinatal period, the other nine tumours in children between the ages of 7 and 16 years. The most frequent anatomical sites were the ovary (6 tumours), sacrococcygeal region (4), testis (4) and mediastinum (3). In contrast to most of the tumours of other localizations, immature ovarian teratomas did not occur in children under 7 years of age (in four cases in association with gliomatosis peritonei). The immature tissue components of the tumours were mostly neuroectodermal structures. Eight tumour specimens showed grade 1, four grade 2 and nine grade 3 malignancy. Grade 3 tridermal teratomas chiefly occurred in young children, whereas two grade 3 monodermal tumours developed in older children. Immunohistochemical analysis of the neuroectodermal components showed that mature astrocytes contained glial fibrillary acid protein, whereas mature nerve cells, nerve fibres and a few groups of immature cells reacted with an antibody to neuron-specific enolase. Six of the 21 patients died; two were stillborn immature infants, two were premature infants, one died postoperatively and one died of metastatic disease. One patient with metastatic disease was alive. None of the 19 children with tridermal immature teratoma showed distant metastases. Metastatic disease was observed in only two patients with presumptive monodermal malignant teratoma. In early childhood the biological behaviour of immature teratomas is evidently similar to that of mature teratomas (provided that the tumour can be totally excised). In older children malignancy must be assumed when the tumour is located in the ovary and/or grade 3 immaturity is determined.
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PMID:Immature teratomas of childhood. Report of 21 cases. 398 17

The subventricular zone (SVZ) of the lateral ventricle remains mitotically active in the adult mammalian central nervous system (CNS). Recent studies have suggested that this region may contain neuronal precursors (neural stem cells) in adult rodents. A variety of neuronal and glial markers as well as three extracellular matrix (ECM) markers were examined with the hope of understanding factors that may affect the growth and migration of neurons from this region throughout development and in the adult. This study has characterized the subventricular zone of late embryonic, postnatal, and adult mice using several neuronal markers [TuJ1, nicotinamide adenine dinucleotide phosphate diaphorase (NADPH-d), neuron-specific enolase (NSE)], glial markers [RC-2, vimentin, glial fibrillary acidic protein (GFAP), galactocerebroside (Gal-C)], ECM markers [tenascin-C (TN-C), chondroitin sulfate, a chondroitin sulfate proteoglycan termed dermatan sulfate-dependent proteoglycan-1 (DSD-1-PG)], stem-cell marker (nestin), and proliferation-specific marker [bromodeoxyuridine (BrdU)]. TuJ1+ and nestin+ cells (neurons and stem cells, respectively) persist in the region into adulthood, although the numbers of these cells become more sparse as the animal develops, and they appear to be immature compared to the cells in surrounding forebrain structures (e.g., not expressing NSE and having few, if any, processes). Likewise, NADPH-d+ cells are found in and around the SVZ during early postnatal development but become more sparse in the proliferative zone through maturity, and, by adulthood, only a few labeled cells can be found at the border between the SVZ and surrounding forebrain structures (e.g., the striatum), and even smaller numbers of positive cells can be found within the adult SVZ proper. BrdU labeling also seems to decrease significantly after the first postnatal week, but it still persists in the SVZ of adult animals. The disappearance of RC-2+ (radial) glia during postnatal development and the persistence of glial-derived ECM molecules such as tenascin and chondroitin sulfate proteoglycans (as well as other "boundary" molecules) in the adult SVZ may be associated with a persistence of immaturity, cell death, and a lack of cell emigration from the SVZ in the adult.
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PMID:Cell and molecular analysis of the developing and adult mouse subventricular zone of the cerebral hemispheres. 854 61

The subependymal zone (SEZ) of the lateral ventricle of adult rodents has long been known to be mitotically active. There has been increased interest in the SEZ, since it has been demonstrated that neuroepithelial stem cells residing there generate neurons in addition to glia in vitro. In the present study, we have examined parasagittal sections of the adult mouse brain using immunocytochemistry for extracellular matrix (ECM) molecules (tenascin and chondroitin sulfate-containing proteoglycans), glial fibrillary acidic protein (GFAP, a cytoskeletal protein prominently expressed by immature and reactive astrocytes), RC-2 (a radial glial and immature astrocyte cytoskeletal marker), TuJ1 (a class III beta-tubulin isoform expressed solely by postmitotic and adult neurons), nestin (a cytoskeletal protein associated with stem cells), neuron-specific enolase, and bromodeoxyuridine (BrdU, which is taken up by dividing cells). Our results demonstrate that a population of young neurons reside within an ECM-rich, GFAP-positive astrocyte pathway from the rostral SEZ all the way into the olfactory bulb. Furthermore, BrdU labeling studies indicate that there is a high level of cell division along the entire length of this path, and double-labeling studies indicate that neurons committed to a neuronal lineage (i.e., TuJ1+) take up BrdU (suggesting they are in the DNA synthesis phase of the cell cycle), again along the entire length of the SEZ "migratory pathway." Thus, the SEZ appears to retain the ability to produce neurons and glia throughout the life of the animal, functioning as a type of "brain marrow." The implications of these findings are discussed in relation to the role that such a glial/ ECM-rich boundary (as seen in the embryonic cortical subplate and other developing areas) may play in: confining the migratory populations and maintaining them in a persistent state of immaturity; facilitating their migration to the olfactory bulb, where they are incorporated into established adult circuitries; and potentially altering SEZ cell cycle dynamics that eventually lead to cell death.
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PMID:Young neurons from the adult subependymal zone proliferate and migrate along an astrocyte, extracellular matrix-rich pathway. 872 38