A deficiency in GALC thus causes a buildup of these fatty acids known as globoid macrophages that destroy oligodendrocytes, thereby inhibiting myelin formation. Because of the presence of globoid cells clustered near white matter, Krabbe disease often goes by the name globoid cell leukodystrophy. Furthermore, new research has shown that Krabbe disease and globoid cell leukodystrophy may be distinct disease entities due to the secretion of inflammatory mediators by natural killer cells in some cases. This research has shown that Natural Killer cells have receptors (TDAG8) for certain glycosphingolipids that build up in an individual with leukodystrophy, again due to insufficient GALC levels, and when bound, target the Natural Killer cells for destruction thereby preventing their cytotoxic effects.
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Globoid cell leukodystrophy PAS - Multinucleated macrophages ("globoid cells") and loss of myelinated fibers in a case of Krabbe's leukodystrophy Like MLD, Krabbe disease is another type of leukodystrophy with autosomal recessive inheritance that is the result of a lysosomal storage disorder. It is due to a deletion in exon 16 of the GALC gene that causes a frameshift mutation leading to a premature stop codon. The GALC gene, found on chromosome 14 at position 31 (14q31), codes for the enzyme beta- galactocerebrosidase (GALC). GALC is a lysosomal enzyme responsible for the catabolism of galactolipids, especially psychosine, that are heavily distributed throughout the brain.
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Glycoside hydrolase family 59 CAZY GH_59 comprises enzymes with only one known activity; galactocerebrosidase (). Globoid cell leukodystrophy (Krabbe disease) is a severe, autosomal recessive disorder that results from deficiency of galactocerebrosidase (GALC) activity. GALC is responsible for the lysosomal catabolism of certain galactolipids, including galactosylceramide and psychosine.
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Eucalyptus globoidea was first formally described in 1927 by William Blakely who published the description in Journal and Proceedings of the Royal Society of New South Wales. The specific epithet (globoidea) is derived from the Latin word globoideus meaning "globoid", referring to the shape of the fruit.
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A variety of crystalloids and globoids. A globoid is a spherical crystalline inclusion in a protein body found in seed tissues that contains phytate and other nutrients for plant growth. These are found in several plants, including wheat and the genus Cucurbita. These nutrients are eventually completely depleted during seedling growth.
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Tumors found in the external third of the sphenoid are of two types: en-plaque and globoid meningiomas. En plaque meningiomas characteristically lead to slowly increasing proptosis with the eye angled downward. Much of this is due to reactive orbital hyperostosis. With invasion of the tumor into the orbit, diplopia is common.
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The company name was Nyegaard & Co. from 1890 until 1986 when it was changed to Nycomed. In 1913 it started producing generic drugs, among these Globoid (copy of Aspirin). In 1969, the revolutionary radiocontrast agent Amipaque was discovered, starting a long process of internationalisation. The next generation product Omnipaque made the company highly successful.
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Microscopically, Lactarius species have elliptical, rarely globoid spores with amyloid ornamentation in the form of more or less prominent warts or spines, connected by ridges, like other members of the family Russulaceae. The trama (flesh) contains spherical cells that cause the brittle structure. Unlike Russula, Lactarius also have lactiferous, i.e. latex-carrying hyphae in their trama.
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Krabbe disease (KD) (also known as globoid cell leukodystrophy or galactosylceramide lipidosis) is a rare and often fatal lysosomal storage disease that results in progressive damage to the nervous system. KD involves dysfunctional metabolism of sphingolipids and is inherited in an autosomal recessive pattern. The disease is named after the Danish neurologist Knud Krabbe (1885-1965).
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An uncommon but severe breed-specific skin condition, hyperplastic dermatosis, may affect West Highland White Terriers, both juveniles and adults dogs. Affected dogs can suffer from red hyperpigmentation, lichenification, and hair loss. In the initial stages, this condition can be misdiagnosed as allergies or less serious forms of dermatitis. An inherited genetic problem that exists in the breed is globoid cell leukodystrophy.
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Patients with globoid meningiomas often present only with signs of increased intracranial pressure. This leads to various other symptoms including headache and a swollen optic disc. The differential diagnosis for sphenoid wing meningioma includes other types of tumors such as optic nerve sheath meningioma, cranial osteosarcoma, metastases, and also sarcoidosis. Following the physical exam, the diagnosis is confirmed with neuro-imaging.
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High concentration of psychosine in dried blood spots may also be identified as a marker for Krabbe disease. A 2011 study discovered that individuals with Krabbe disease, more so in later-onset individuals, tend to have an abnormal increase in CSF protein concentration. The disease may be diagnosed by its characteristic grouping of certain cells (multinucleated globoid cells), nerve demyelination and degeneration, and destruction of brain cells. Special stains for myelin (e.g.
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In 1902 Krebs invented the automatic diaphragm carburettor which gave cars continuous power during acceleration by providing a constant air- fuel ratio at all times; this also led to dramatic improvement in fuel economy. In 1906 Krebs traveled to the United States to plead in the Selden Case, associated with Henry Ford. The 1906 Krebs testimony in the Selden case Krebs introduced many improvements in car design: the steering wheel (1898), non-reversible steering (1898), engine balance (1898), nickel steel alloys and other special steel alloys (1901), the shock absorber (1906), multi-disc clutch (1907), the electric brake dynamometer for testing high performance engines (1905), the enveloping (globoid) Worm gear differential (1915).
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Oligodendrocyte precursor cells and neural stem cells have been transplanted successfully and have shown to be healthy a year later. Fractional anisotropy and radial diffusivity maps showed possible myelination in the region of the transplant. Induced pluripotent stem cells, oligodendrocyte precursor cells, gene correction, and transplantation to promote the maturation, survival, and myelination of oligodendrocytes seem to be the primary routes for possible treatments. For three types of leukodystrophies (X-linked adrenoleukodystrophy (X-ALD), metachromatic leukodystrophy (MLD) and Krabbe Disease (globoid cell leukodystrophy - GLD), gene therapy using autologous hematopoietic stem cells to transfer the disease gene with lentiviral vectors have shown to be successful and are currently being used in clinical trials for X-ALD and MLD.
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