Abstract:
:A mutation in the Vps33a gene causes Hermansky-Pudlak Syndrome (HPS)-like-symptoms in the buff (bf) mouse mutant. The encoded product, Vps33a, is a member of the Sec1 and Class C multi-protein complex that regulates vesicle trafficking to specialized lysosome-related organelles. As Sec1 signaling pathways have been implicated in pre-synaptic function, we examined brain size, cerebellar cell number and the behavioral phenotype of bf mutants. Standardized behavioral tests (SHIRPA protocols) demonstrated significant motor deficits (e.g., grip strength, righting reflex and touch escape) in bf mutants, worsening with age. Histological examination of brain revealed significant Purkinje cell loss that was confirmed with staining for calbindin, a calcium binding protein enriched in Purkinje cells. This pathologic finding was progressive, as older bf mutants (13-14 months) showed a greater attrition of neurons, with their cerebella appearing to be particularly reduced (approximately 30%) in size relative to those of age-matched-control cohorts. These studies suggest that loss of Purkinje neurons is the most obvious neurological atrophy in the bf mutant, a structural change that generates motor coordination deficits and impaired postural phenotypes. It is conceivable therefore that death of cerebellar cells may also be a clinical feature of HPS patients, a pathological event which has not been reported in the literature. In general, the bf mutant may be a potentially new and useful model for understanding Purkinje cell development and function.
journal_name
Brain Resjournal_title
Brain researchauthors
Chintala S,Novak EK,Spernyak JA,Mazurchuk R,Torres G,Patel S,Busch K,Meeder BA,Horowitz JM,Vaughan MM,Swank RTdoi
10.1016/j.brainres.2009.02.035subject
Has Abstractpub_date
2009-04-17 00:00:00pages
18-28eissn
0006-8993issn
1872-6240pii
S0006-8993(09)00342-4journal_volume
1266pub_type
杂志文章相关文献
BRAIN RESEARCH文献大全abstract::The suprachiasmatic nucleus of the hypothalamus receives dense serotonergic projections from the raphe nuclei. This input may be involved in the transmission of photic information with activation of 5-HT receptors at night having a photo-mimetic effect. 5-HT2C receptors have been implicated in this role, with 5-HT2C r...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/j.brainres.2008.02.091
更新日期:2008-05-13 00:00:00
abstract::Sensory fibers trapped in nerve-end neuromas become abnormally excitable, and produce an ectopic discharge which is believed to contribute to paresthesias and pain associated with chronic nerve injury in man. Here we report that stimulation of injured nerves can alter this discharge, directly by antidromic invasion of...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(87)90275-7
更新日期:1987-07-07 00:00:00
abstract::Melatonin is a hormone that mostly produced from the pineal gland, and it performs as a strong neuroprotectant to both neuron and glial cells against methamphetamine (METH)-induced neurotoxicity. Recently, it has been found that METH also damages the blood brain barrier (BBB) structure and function. However, the prote...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/j.brainres.2016.08.045
更新日期:2016-11-01 00:00:00
abstract::The role of the cerebellum and the red nucleus in the conditioned eyeblink response was assessed, using a combination of reversible lesions and multiple-unit extracellular recording in the awake, behaving rabbit. Lesion, recording, and stimulation experiments have indicated that both of these structures are involved i...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(90)90351-b
更新日期:1990-12-24 00:00:00
abstract::In the sea hare, Aplysia californica, consummatory feeding behavior is selectively abolished by bilateral crushes of the cerebral-buccal connectives and recovers by postlesion day 13. Recovered biting responses are initially weak and increase in magnitude gradually with time. The lesions do not affect appetitive feedi...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(92)91217-3
更新日期:1992-07-10 00:00:00
abstract::We have recently reported the existence within chick embryo heart cell conditioned medium (HCM) of two distinct and independently assayable factors. One agent, ciliary neuronotrophic factor (CNTF), supports the in vitro survival of 8-day chick embryo ciliary ganglionic (CG) neurons. The other factor, polyornithine-att...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(81)90105-0
更新日期:1981-02-09 00:00:00
abstract::Dopamine (DA) and noradrenaline (NA) extracellular levels have been measured by microdialysis in the medial frontal cortex (MFC), nucleus accumbens (NAc) and caudate-putamen (CP) under baseline conditions in awake and halothane-anaesthetized rats, and after application of three types of stimuli which are likely to act...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(92)90711-h
更新日期:1992-05-29 00:00:00
abstract::The role of cat's primary auditory cortex (AI) in both pattern discrimination and sound localization has been demonstrated by observing that ablations of it disrupt these functions. This research studied effective connectivity variations as a function of modifications in the temporal pattern of acoustic stimulation. R...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(88)91542-9
更新日期:1988-05-31 00:00:00
abstract::During the middle of the subjective day, circadian activity rhythms in Syrian hamsters can be phase advanced by a variety of stimuli including microinjection of neuropeptide Y (NPY) or muscimol into the suprachiasmatic nucleus (SCN). It is not known, however, if these treatments shift activity rhythms by acting direct...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/s0006-8993(97)00831-7
更新日期:1997-10-24 00:00:00
abstract::The response of neurons in the preoptic and adjacent regions of the brain to intracarotid injection of hyperosmotic solutions was studied in 22 anesthetized cats. NaCl (0.5 M) caused a brisk acceleration of frequency lasting over 100 s in 120 of 207 units (58%). Glucose (2 M) and sucrose (2 M) had similar but smaller ...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(85)91026-1
更新日期:1985-10-21 00:00:00
abstract::We have previously identified a cell senescence-related, detergent-insoluble cytoplasmic protein referred to as terminin, and have presented the development-dependent modification of terminin polypeptides in rat brain. In the present study, we characterize biochemically and immunocytochemically terminin expression in ...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(94)91679-9
更新日期:1994-05-02 00:00:00
abstract::Intracerebral injection of kainic acid (KA) in rat brain was previously found to cause altered immunohistochemical recognition of connexin-43 (Cx43) epitopes (epitope masking) with different sequence-specific antibodies against this gap junction protein. We demonstrate here that similar alterations occur when nitrocel...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(95)00337-p
更新日期:1995-06-12 00:00:00
abstract::In alert monkeys, some prefrontal neurons located in the superior dorsolateral area were activated to acoustic stimuli delivered in a restricted range of directions with respect to the animal's head. The effective direction was usually contralateral to the animal. The function of the auditory neurons in connection to ...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(84)91434-3
更新日期:1984-04-30 00:00:00
abstract::The tumor promoting compound 12-O-tetradecanoyl-phorbol-13-acetate (TPA) specifically binds to and activates protein kinase C (PKC). This enzyme is widely distributed in the brain. It plays a pivotal role in transmembrane signalling. Arachidonic acid conversion is a common cellular response to membrane perturbation. W...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(87)90461-6
更新日期:1987-08-11 00:00:00
abstract::The discovery of glucagon biosynthesis and receptors within mammalian brain has led one to suspect a neurotransmitter role for glucagon. In order to address this hypothesis in birds, we investigated the existence of glucagon receptors in duck brain by radioligand binding on fresh tissue sections and radioautography. S...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(94)90469-3
更新日期:1994-11-07 00:00:00
abstract::(1) The possibility that water intake elicited by the administration of angiotensin-II to the preoptic region (POA), subfornical organ and anterior third ventricle is mediated by separate neural systems was investigated in 58 male Wistar rats using electrolytic lesion techniques. (2) Lesions of the midlateral hypothal...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(76)90189-x
更新日期:1976-05-28 00:00:00
abstract::The effect of increasing amounts of amyloid beta-protein (Abeta) on the number of neurons in the striatum was investigated in APPswe/PS1DeltaE9 transgenic mice. This mouse model of Alzheimer's disease-like amyloidosis displays an increased expression of Abeta in the brain with age. On the basis of reports of disruptio...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/j.brainres.2009.02.025
更新日期:2009-04-17 00:00:00
abstract::The effects of the 8-alpha-amino-ergoline CU 32-085 on central dopamine neuronal systems was investigated. Two h after administration of CU 32-085 a slight increase of dopamine levels was observed in the nucleus caudatus-putamen. Radioligand binding studies in vitro have shown that CU 32-085 has a low affinity for str...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(85)91044-3
更新日期:1985-03-04 00:00:00
abstract::It has been hypothesized that a disruption of gamma-aminobutyric acid (GABA) receptor-mediated processes may be involved in the pathophysiology of focal epilepsy. This disinhibition hypothesis has been postulated from the results of in vitro experiments of the interictal activity of focal epilepsy. Less is known, howe...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/s0006-8993(00)02209-5
更新日期:2000-05-26 00:00:00
abstract::Spontaneous interictal spikes (SISs) were recorded in the hippocampus in freely behaving rats following hippocampal stimulations that resulted in afterdischarges (ADs). Hippocampal SISs were detected after an average of 5 (range 2-10) daily ADs. The rate of SISs typically increased minutes after a tetanus, and then de...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(90)90472-n
更新日期:1990-04-16 00:00:00
abstract::The lack of the glycine transporter 1 (GlyT1) leads to early postnatal death due to failure of respiratory network activity. Here we demonstrate a segregated expression of GlyT1 on different astroglial cell populations of the ventral respiratory group. In TgN(hGFAP-EGFP) mice a combined immunohistochemical and electro...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/j.brainres.2006.08.089
更新日期:2006-11-13 00:00:00
abstract::Single unit discharges in the superior secondary gustatory nucleus of the carp, Cyprinus carpio L., were studied electrophysiologically in response to chemical stimulation of the external chemoreceptors of the facial skin surface. Of 36 gustatory neurons recorded, 80.6% were facilitated by taste stimuli and 19.4% were...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(81)90184-0
更新日期:1981-07-27 00:00:00
abstract::Systemic administration of 3-nitropropionic acid (3-NP) results in striatal atrophy by irreversibly inhibiting the citric acid cycle, and thereby resulting in cellular ATP depletion. The neuropathological outcome following 3-NP injections is thought to resemble that seen in Huntington's disease (HD) [1]. The current s...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(94)90085-x
更新日期:1994-05-23 00:00:00
abstract::A diffusion model is described here, which allows for the estimations of drug concentration changes in porous media, such as in slice tissues of the central nervous system (CNS) bathed in unstirred solutions following abrupt changes of drug concentration. This model may be used for the interpretation of data obtained ...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(87)91523-x
更新日期:1987-12-22 00:00:00
abstract::A site-specific antibody against the principal gap junctional protein in heart (connexin 43) was used to determine immunohistochemically the cellular localization of this protein in rat brain. Structures labelled with the antibody included gap junctional membranes between glial, ependymal, pial and arachnoid cells as ...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(90)90415-8
更新日期:1990-02-05 00:00:00
abstract::In normal subjects, electrical stimulation of trigeminal mucosal afferents (lingual nerve - V3) can elicit a short latency (12.5+/-0. 3 ms; mean+/-S.D.) reflex response in the ipsilateral genioglossus muscle (Maisonobe et al., Reflexes elicited from cutaneous and mucosal trigeminal afferents in normal human subjects. ...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/s0006-8993(00)02177-6
更新日期:2000-05-02 00:00:00
abstract::The effect of chronic ethanol administration on the density of basket cells in the dentate gyrus of mice selectively bred for their sensitivity to acute ethanol exposure (long-sleep, LS and short-sleep, SS) was assessed in two experiments. In addition, the effect of chronic ethanol on the density of dentate granule ce...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(88)90369-1
更新日期:1988-09-20 00:00:00
abstract::Calretinin and calbindin-D28k are two calcium-binding proteins which are present in separate populations of interneurons in cerebral cortex and hippocampus. To identify these cells with the populations expressing different transmitters, two-colour immunofluorescence was done with antibodies against the calcium-binding...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(92)91439-l
更新日期:1992-07-31 00:00:00
abstract::The modulation of serotonin (5-HT) release by opioid receptors in the hippocampus of the awake, unrestrained rat was evaluated by use of in vivo microdialysis. The hippocampus was perfused with Ringer's solution (2 microliters/min), and extracellular levels of 5-HT and its major metabolite, 5-hydroxyindoleacetic acid ...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(93)90456-w
更新日期:1993-06-04 00:00:00
abstract::The developmental time course and life span of the human brain are different from those of laboratory animals. These variations may be the reflection of metabolic differences of the neurons between different species. Using immunocytochemistry, we show that pyramidal neurons accumulate advanced glycosylation end produc...
journal_title:Brain research
pub_type: 杂志文章
doi:10.1016/0006-8993(94)90156-2
更新日期:1994-04-04 00:00:00