Biomaterial Database

Glutamate dehydrogenase in olivopontocerebellar atrophies: leukocytes, fibroblasts, and muscle mitochondria. 1986

G Finocchiaro, and F Taroni, and S Di Donato

Glutamate dehydrogenase (GDH) activity was 68% of control values in leukocyte homogenates of 11 patients with dominant olivopontocerebellar atrophies (OPCA) and 46% in muscle mitochondria of 4 patients with dominant OPCA. In three patients with recessive OPCA and in one sporadic patient, muscle GDH was lower than in controls. However, muscle GDH activity was normal in one of two dominant patients in the same family and decreased in the other, and patients' activities overlapped with lower control values. Plasma glutamate levels were significantly higher in dominant patients than in controls after glutamate challenge. GDH activity may be partially altered at the mitochondrial level in a subgroup of OPCA patients.

UI	MeSH Term	Description	Entries
D007962	Leukocytes	White blood cells. These include granular leukocytes (BASOPHILS; EOSINOPHILS; and NEUTROPHILS) as well as non-granular leukocytes (LYMPHOCYTES and MONOCYTES).	Blood Cells, White,Blood Corpuscles, White,White Blood Cells,White Blood Corpuscles,Blood Cell, White,Blood Corpuscle, White,Corpuscle, White Blood,Corpuscles, White Blood,Leukocyte,White Blood Cell,White Blood Corpuscle
D008297	Male		Males
D008931	Mitochondria, Muscle	Mitochondria of skeletal and smooth muscle. It does not include myocardial mitochondria for which MITOCHONDRIA, HEART is available.	Sarcosomes,Mitochondrion, Muscle,Muscle Mitochondria,Muscle Mitochondrion,Sarcosome
D009847	Olivary Nucleus	A brainstem nuclear complex. in the hindbrain, also referred to as the olivary body. The olivary nuclear complex is a part of the MEDULLA OBLONGATA and the PONTINE TEGMENTUM. It is involved with motor control and is a major source of sensory input to the CEREBELLUM.	Basal Nucleus, Olivary,Nucleus Basalis, Olivary,Olivary Body,Olivary Complex,Olivary Nuclei,Complex, Olivary,Nucleus, Olivary,Nucleus, Olivary Basal,Olivary Basal Nucleus,Olivary Bodies
D011149	Pons	The front part of the hindbrain (RHOMBENCEPHALON) that lies between the MEDULLA and the midbrain (MESENCEPHALON) ventral to the cerebellum. It is composed of two parts, the dorsal and the ventral. The pons serves as a relay station for neural pathways between the CEREBELLUM to the CEREBRUM.	Pons Varolii,Ponte,Pons Varolius,Pontes,Varolii, Pons,Varolius, Pons
D001927	Brain Diseases	Pathologic conditions affecting the BRAIN, which is composed of the intracranial components of the CENTRAL NERVOUS SYSTEM. This includes (but is not limited to) the CEREBRAL CORTEX; intracranial white matter; BASAL GANGLIA; THALAMUS; HYPOTHALAMUS; BRAIN STEM; and CEREBELLUM.	Intracranial Central Nervous System Disorders,Brain Disorders,CNS Disorders, Intracranial,Central Nervous System Disorders, Intracranial,Central Nervous System Intracranial Disorders,Encephalon Diseases,Encephalopathy,Intracranial CNS Disorders,Brain Disease,Brain Disorder,CNS Disorder, Intracranial,Encephalon Disease,Encephalopathies,Intracranial CNS Disorder
D002531	Cerebellum	The part of brain that lies behind the BRAIN STEM in the posterior base of skull (CRANIAL FOSSA, POSTERIOR). It is also known as the "little brain" with convolutions similar to those of CEREBRAL CORTEX, inner white matter, and deep cerebellar nuclei. Its function is to coordinate voluntary movements, maintain balance, and learn motor skills.	Cerebella,Corpus Cerebelli,Parencephalon,Cerebellums,Parencephalons
D005260	Female		Females
D005347	Fibroblasts	Connective tissue cells which secrete an extracellular matrix rich in collagen and other macromolecules.	Fibroblast
D005969	Glutamate Dehydrogenase	An enzyme that catalyzes the conversion of L-glutamate and water to 2-oxoglutarate and NH3 in the presence of NAD+. (From Enzyme Nomenclature, 1992) EC 1.4.1.2.	Dehydrogenase, Glutamate