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At least 1,783 records · Page 99Linked to original sources
PubMed2001

An instructive function for Notch in promoting gliogenesis in the zebrafish retina.

The Gal4-UAS technique has been used to misexpress a constitutively active Notch receptor variant (notch1a-intra) in the developing zebrafish retina. This is the first study to use this technique to misexpress genes and assess their function in neural development of the zebrafish. Expression of activated Notch1a either ubiquitously, driven by a heat-shock70 promoter, or in a spatially regulated manner, controlled by the deltaD promoter, causes a block in neuronal differentiation that affects all cell types. Developing cells take on either a glial fate or remain undifferentiated. A large number of cells eventually undergo apoptosis. These phenotypic effects of activated Notch1a are expressed cell autonomously. Cells within central regions of the retina adopt a glial fate if they express activated Notch1a in a time window that extends from 27 to 48 hours postfertilization. This period corresponds mainly to the time of origin of ganglion cells in the normal retina. Activation of notch1a at later stages results in defects in cell type specification that remain restricted to the ciliary marginal zone, whereas neuronal types are specified normally within the central region. These observations indicate that glial differentiation is initiated by Notch1a-intra expressing cells, which become postmitotic in the same time window. Our results strongly suggest that Notch1a instructs a certain cell population to enter gliogenesis, and keeps the remaining cells in an undifferentiated state. Some or all of these cells will eventually succumb to apoptosis.

Animals↗
PubMed1977

Abnormal urogenital organs occurring spontaneously in inbred ACI and Kyoto-notched rats.

Spontaneous malformations of urogenital organs found in inbred rat foetuses of ACI/NHok strains were further investigated with a postnatal survey involving other inbred strains. A new type of malformation occurred in Kyoto-notched rats. The present findings suggested that the malformations have different hereditary characteristics caused by different maldevelopmental mechanisms and are probably due to recessive polygenes.

Animals↗
PubMed2005

Bone deformity showing a deep coronoid notch of the mandible in a patient with neurofibromatosis type 1.

We present a case of a 10-year-old girl with neurofibromatosis type 1 related skeletal dysplasia. In the present case, a unilocular radiolucency at the left coronoid notch visible on a panoramic radiograph was suspected to be a neurofibroma. However, only the presence of bone deformities was observed on CT. Fat-like tissue adjacent to the bone deformities inside the left mandibular ramus was revealed, and the presence of neurofibroma was unequivocally denied on the MR images. However, biopsy of the lesion suggested neurofibroma. The diagnostic imaging 18 months after the first visit did not revealed a tumourous mass. However, careful follow up is required even though we could not prove the presence the lesion on the basis of imaging.

Biopsy↗
PubMed2005

Rang retractors for sciatic notch exposure during pelvic osteotomy.

We present simple but effective retractors used in pairs to expose the sciatic notch during Salter innominate osteotomy. We have found them to be useful for a wide range of procedures requiring similar exposure. We present them here in tribute to the memory of the designer Mercer Rang.

Equipment Design↗
PubMed2003

Deep-notch, ultracompact long-period grating in a large-mode-area photonic crystal fiber.

An extremely short long-period grating (LPG) with strong resonance has been developed in a large-mode-area photonic crystal fiber (PCF) by use of the heat source of a CO2 laser. We believe that such a LPG in pure silica PCF is the first example to be obtained with the point-by-point technique. The fabrication method is simple and repeatable. The resulting LPG has 8 periods, written by a CO2 laser, within a 2.8-mm length of fiber, which yields a deep notch of core-cladding mode coupling of -31.5 dB at the telecommunication wavelength of 1529.2 nm, with a FWHM of approximately 0.7 nm. The principal advantages of this LPG are that it is practical, cost effective, and compact.

Journal Article↗
PubMed1976

Development of rib notching after a cava-pulmonary anastomosis.

A patient with tetralogy of Fallot and mitral regurgitation developed unilateral rib notching on the right side secondary to a palliative cava-pulmonary anastomosis. After the operation, decreased perfusion of the right upper and middle lobes stimulated the development of collateral vessels from the systemic circulation to the right pulmonary artery. To our knowledge, this is the first time that this has been described.

Adolescent↗
PubMed2005

Deregulated NOTCH signaling in acute T-cell lymphoblastic leukemia/lymphoma: new insights, questions, and opportunities.

Recent work has shown that the majority of human acute T-cell lymphoblastic leukemias and lymphomas (T-ALL) have gain-of-function mutations in NOTCH1, a type I transmembrane receptor that normally signals through a gamma-secretase-dependent mechanism that relies on ligand-induced regulated intramembranous proteolysis. Cleavage by gamma-secretase releases the intracellular domain of NOTCH1 (ICN1), permitting it to translocate to the nucleus and form a short-lived transcriptional activation complex that is essential for normal T-cell development. Two types of mutations are prevalent in human T-ALL: extracellular domain mutations that increase ICN1 production and C-terminal mutations that sustain ICN1 action. Inhibitors of ICN1 production and activity abrogate the growth of established T-ALL cell lines, and a clinical trial of a NOTCH pathway inhibitor in patients with refractory T-ALL has opened recently. These insights raise a number of new questions relevant to T-ALL pathogenesis and offer exciting opportunities for rational targeted therapy.

Dimerization↗
PubMed2006

Nuclear receptor regulation gears up another Notch.

In this perspective we describe examples of crosstalk between nuclear receptors (NRs) and Notch signaling by means of direct functional interactions between components of both pathways. This crosstalk may provide eukaryotic organisms with molecular mechanisms for the coordination of llong-distance endocrine signals with cell-to-cell juxtacrine communication.

Journal Article↗
PubMed1997

Magnetic resonance imaging of sequelae of temporal lobe herniation secondary to traumatic acute subdural hematoma: Kernohan's notch and posterior cerebral artery territory infarctions contralateral to the supratentorial lesion--case report.

A 16-year-old girl presented with left hemiparesis and right oculomotor nerve paresis due to temporal lobe herniation resulting from a traumatic acute subdural hematoma secondary to a head injury. Computed tomography revealed a subdural hematoma in the right frontotemporal regions and midline structures shifted to the left. She became comatose and underwent an emergency operation. The hemiparesis and newly manifested homonymous hemianopsia on the right side persisted after surgery. Postoperative magnetic resonance imaging showed Kernohan's notch in the cerebral peduncle and infarctions in the occipital lobe and posterolateral part of the thalamus on the left side, contralateral to the supratentorial lesion.

Adolescent↗
PubMed1976

The notched articular process of C7 (dorsalization of C7).

The frequency of variation from the standard in the articular processes of C7 is very high (43.9 percent). These variations are: (1) A notch on the back of the superior articular facet. (2) Elongation the the "articular pillary" so that the posterior edge of the inferior articular process of C7 lies backward in relation to the posterior edges of the other cervical articular process. (3) A combination of both. Item 1 should not be mistaken for a fracture and item 2 should not be mistaken for a dislocation. These variants represent a partial dorsalization of C7.

Age Factors↗
PubMed1990

Thresholds for short-latency auditory-evoked potentials to tones in notched noise in normal-hearing and hearing-impaired subjects.

The thresholds for the short-latency auditory evoked potentials (SLAEPs) to short-duration tones presented in notched-noise masking were evaluated in 20 normal-hearing and 20 hearing-impaired subjects. The differences (dB) between these thresholds (dB nHL) and the pure-tone behavioral thresholds (dB HL) across all 40 subjects were 11.6, 6.1, 6.3 and 0.8 dB for 500, 1,000, 2,000 and 4,000 Hz, respectively. These differences were significantly smaller for the hearing-impaired subjects than for the normal-hearing subjects. Ninety-eight percent of the SLAEP threshold estimations were within 30 dB of the subjects' pure-tone behavioral thresholds and 91% were within 20 dB.

Adolescent↗
PubMedDate not supplied

Frequency-specific brainstem responses to bone-conducted tone pulses masked by notched noise.

As in the case of auditory brainstem responses (ABRs) to air-conducted stimuli, recording of frequency-specific ABRs to bone-conducted stimuli needs adequate masking of those parts of the basilar membrane that are not to contribute to the ABR. The present study shows that tone-pulse stimulation with notched noise-masking can be realized via a bone vibrator after its frequency response has been flattened. The latency-intensity curves for 4, 2, 1, and 0.5 kHz run approximately parallel, indicating the ABR to be indeed frequency-specific. As adequate air-conducted masking of the nontest ear can produce cross-masking, the use of an insert earphone is proposed. Because of its higher interaural attenuation, a higher masking level can be applied to the nontest ear.

Bone Conduction↗
PubMed1996

Genetic interactions among vestigial, hairy, and Notch suggest a role of vestigial in the differentiation of epidermal and neural cells of the wing and halter of Drosophila melanogaster.

In this paper we describe the results of genetic analysis of the vestigial locus by studying its interactions with hairy and Notch loci in Drosophila melanogaster. Different vestigial alleles in homo- and heterozygous combination with different hairy alleles show synergism in increasing both cell death and formation of ectopic bristles and produce ectopic veins. Interactions between N and vg also show synergism in increasing cell death and formation of ectopic bristles. Only synergism in cell death is seen between h and N. The interactions indicate that vg product plays a role in the differentiation of epidermal and neural cells of the wing disc by interacting with N and h products either directly or indirectly. Mechanisms of molecular interactions among the three loci are discussed.

Animals↗
PubMed1991

Removal of the roof of the external auditory meatus in approaching the tentorial notch through a low temporal craniotomy. Technical note.

Improved access to the tentorial notch can be obtained by removal of the roof of the external auditory meatus in association with a low temporal craniotomy. This approach decreases temporal lobe retraction and the risk of venous infarction. This method was perfected in the surgical laboratory on five cadavers and was successfully performed in a patient with a giant aneurysm of the posterior cerebral artery.

Craniotomy↗
PubMed2004

Attenuation of experimental autoimmune encephalomyelitis and nonimmune demyelination by IFN-beta plus vitamin B12: treatment to modify notch-1/sonic hedgehog balance.

Interferon-beta is a mainstay therapy of demyelinating diseases, but its effects are incomplete in human multiple sclerosis and several of its animal models. In this study, we demonstrate dramatic improvements of clinical, histological, and laboratory parameters in in vivo mouse models of demyelinating disease through combination therapy with IFN-beta plus vitamin B(12) cyanocobalamin (B(12)CN) in nonautoimmune primary demyelinating ND4 (DM20) transgenics, and in acute and chronic experimental autoimmune encephalomyelitis in SJL mice. Clinical improvement (p values <0.0001) was paralleled by near normal motor function, reduced astrocytosis, and reduced demyelination. IFN-beta plus B(12)CN enhanced in vivo and in vitro oligodendrocyte maturation. In vivo and in vitro altered expression patterns of reduced Notch-1 and enhanced expression of sonic hedgehog and its receptor were consistent with oligodendrocyte maturation and remyelination. IFN-beta-B(12)CN combination therapy may be promising for the treatment of multiple sclerosis.

Acute Disease↗
PubMedDate not supplied

Greater sciatic notch in sex differentiation in Nigerian skeletal samples.

A total of 150 X-ray films (A-P view) of the pelvis of adult (90 male and 60 female) Nigerians were used to measure the width, depth, posterior segment, total and posterior angles of the greater sciatic notch. Index 1 was calculated by dividing the greatest depth OC by the greatest width AB and multiplying by 100. Index 2 was calculated by diving the posterior segment OB by the greatest width (AB) and multiplying by 100. These indices were used to study the sex differences in the hip bones of these Nigerians. Demarking points of these parameters and indices were worked out to determine sex. The width, depth, total angle and index 1 were insignificant in sex determination. Posterior angle and index 2 were found to be the most useful assigning sex to 74.5% of the male and 90% of the female bones.

Adolescent↗
PubMedDate not supplied

[Report of a patient with CADASIL having a novel missense mutation of the Notch 3 gene--association with alopecia and lumbar herniated disk].

We report a 52-year-old man with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) presenting dementia, alopecia and lumbar herniated disk. He had an episode of stroke and migraine-like headache lasting for 5 minutes. A lot of members had cerebral infarction in this family. Brain magnetic resonance imaging demonstrated, on T2-weighted images, numerous hyperintense lesions suggestive of small infarcts in the basal ganglia and diffuse hyperintense lesions in the cerebral white matter. The clinical symptoms, the family history and the MRI findings suggested the diagnosis of CADASIL. However, the patient also showed alopecia and lumbar herniated disk, both are characteristic features of cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL). The DNA analysis of the Notch 3 gene identified a novel missense mutation Cys174Phe in this patient. Our case report indicated the importance of the DNA analysis for the diagnosis of CADASIL.

Alopecia↗
PubMed2001

Comparison of males' and females' ratios of anterior-cruciate-ligament width to femoral-intercondylar-notch width: a cadaveric study.

The relative incidence of anterior cruciate ligament (ACL) injuries has been reported to be significantly higher in females than in males. Although many hypotheses have been proposed for this discrepancy, no conclusive explanation has reached consensus opinion. The purpose of this study was to evaluate and compare fresh-frozen cadaveric knee specimens to determine whether there is a significant difference between males' and females' ratios of ACL width to femoral intercondylar notch (FIN) width. We evaluated 15 male knees and 11 female knees. Data regarding sex, age, side (right or left), and ACL and FIN widths were obtained for all specimens. Age of male specimens ranged from 48 to 84 years (mean, 63.9 y); age of female specimens ranged from 33 to 96 years (mean, 69.9 y). Mean ACL width, mean FIN width, and their ratios were calculated. In the male specimens, mean ACL width was 10.59 mm (SD, 1.30 mm), mean FIN width was 20.18 mm (SD, 2.20 mm), and mean ACL:FIN width ratio was .526 +/- 0.047 (52.6% +/- 4.7% of FIN width); in the female specimens, mean ACL width was 8.09 mm (SD, 1.12 mm), mean FIN width was 20.50 mm (SD, 1.69 mm), and mean ACL: FIN width ratio was .393 +/- 0.03 (39.3% +/- 3% of FIN width). Results showed statistically significant differences between males' and females' ACL widths (P < .001) and between their ACL: FIN width ratios (P< .001) but not between their FIN widths. The significant difference between males' and females' ACL widths may be related to the general size differences between the groups. It has been speculated that female athletes' smaller ACLs may predispose them to ACL ruptures. Perhaps more significant is the difference between males' and females' ACL: FIN width ratios, which are significantly smaller in females compared with males. This may be directly related to the cause of more ACL ruptures in females. Further studies, including a good mechanical model, will help elucidate whether this anatomic discrepancy correlates clinically.

Adult↗