José A. Armengol

José A. Armengol
Universidad Pablo de Olavide | UPO · Department of Physiology, Anatomy and Cellular Biology

PhD, MD

About

73
Publications
4,856
Reads
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1,079
Citations
Citations since 2017
17 Research Items
291 Citations
20172018201920202021202220230102030405060
20172018201920202021202220230102030405060
20172018201920202021202220230102030405060
20172018201920202021202220230102030405060
Additional affiliations
February 2010 - December 2015
Universidad Pablo de Olavide
Position
  • Head of Department
October 2004 - present
Universidad Pablo de Olavide
Position
  • Professor of Human Anatomy and Embryology
October 1981 - September 2004
Universidad de Sevilla
Position
  • Professor (Full)
Education
October 1975 - July 1983
School of Medicine. University of Seville
Field of study
  • Medicine

Publications

Publications (73)
Article
Mitochondrial diseases are a heterogeneous group of rare genetic disorders caused by mutations in nuclear or mitochondrial DNA (mtDNA). These diseases are frequently multisystemic, although mainly affect tissues that require large amounts of energy such as the brain. Mutations in mitochondrial transfer RNA (mt-tRNA) lead to defects in protein trans...
Article
Full-text available
In the last decade, new non-apoptotic roles have been ascribed to apoptotic caspases. This family of proteins plays an important role in the sculpting of the brain in the early stages of development by eliminating excessive and nonfunctional synapses and extra cells. Consequently, impairments in this process can underlie many neurological and menta...
Article
Full-text available
Background: PLA2G6-Associated Neurodegeneration (PLAN) is a rare neurodegenerative disease with autosomal recessive inheritance, which belongs to the NBIA (Neurodegeneration with Brain Iron Accumulation) group. Although the pathogenesis of the disease remains largely unclear, lipid peroxidation seems to play a central role in the pathogenesis. Curr...
Article
Full-text available
Background PLA2G6-Associated Neurodegeneration (PLAN) is a rare neurodegenerative disease with autosomal recessive inheritance, which belongs to the NBIA (Neurodegeneration with Brain Iron Accumulation) group. Although the pathogenesis of the disease remains largely unclear, lipid peroxidation seems to play a central role in the pathogenesis. Curre...
Article
The HERC protein family is one of three subfamilies of Homologous to E6AP C-terminus (HECT) E3 ubiquitin ligases. Six HERC genes have been described in humans, two of which encode Large HERC proteins -HERC1 and HERC2- with molecular weights above 520 kDa that are constitutively expressed in the brain. There is a large body of evidence that mutation...
Article
Neurodegeneration with brain iron accumulation (NBIA) is a group of inherited neurologic disorders in which iron accumulates in the basal ganglia resulting in progressive dystonia, spasticity, parkinsonism, neuropsychiatric abnormalities, and optic atrophy or retinal degeneration. The most prevalent form of NBIA is pantothenate kinase-associated ne...
Preprint
Full-text available
Diversity within microglia, the resident brain immune cells, is reported. Whether microglial subsets constitute different subtypes with intrinsic properties and unique functions has not been fully elucidated. Here, we describe a microglial subtype characterized by the expression of the enzyme Arginase-1, i.e. Arg1 + microglia, which is found predom...
Article
Full-text available
Mutations in the human HERC1 E3 ubiquitin ligase protein develop intellectual disability. The tambaleante (tbl) mouse carries a HERC1 mutation characterized by cerebellar ataxia due of adult cerebellar Purkinje cells death by extensive autophagy. Our previous studies demonstrated that both the neuromuscular junction and the peripheral nerve myelin...
Article
Full-text available
Neuromuscular disorders (NMDs) affect 1 in 3000 people worldwide. There are more than 150 different types of NMDs, where the common feature is the loss of muscle strength. These disorders are classified according to their neuroanatomical location, as motor neuron diseases, peripheral nerve diseases, neuromuscular junction diseases, and muscle disea...
Article
Full-text available
HERC1 is a ubiquitin ligase protein, which, when mutated, induces several malformations and intellectual disability in humans. The animal model of HERC1 mutation is the mouse tambaleante characterized by: (1) overproduction of the protein; (2) cerebellar Purkinje cells death by autophagy; (3) dysregulation of autophagy in spinal cord motor neurons,...
Article
Full-text available
Critical periods of synaptic plasticity facilitate the reordering and refining of neural connections during development, allowing the definitive synaptic circuits responsible for correct adult physiology to be established. Presynaptic spike timing-dependent long-term depression (t-LTD) exists in the hippocampus, which depends on the activation of N...
Article
Full-text available
A missense mutation in HERC1 provokes loss of cerebellar Purkinje cells, tremor, and unstable gait in tambaleante (tbl) mice. Recently, we have shown that before cerebellar degeneration takes place, the tbl mouse suffers from a reduction in the number of vesicles available for release at the neuromuscular junction (NMJ). The aim of the present work...
Article
Full-text available
Tambaleante (tbl/tbl) is a mutant mouse that carries a spontaneous Gly483Glu substitution in the HERC1 (HECT domain and RCC1 domain) E3 ubiquitin ligase protein (HERC1). The tbl/tbl mutant suffers an ataxic syndrome given the almost complete loss of cerebellar Purkinje cells during adult life. More recent analyses have identified alterations at neu...
Article
Full-text available
The spontaneous mutation tambaleante is caused by the Gly483Glu substitution in the highly conserved N terminal RCC1-like domain of the HERC1 protein, which leads to the increase of mutated protein levels responsible for cerebellar Purkinje cell death by autophagy. Until now, Purkinje cells have been the only central nervous neurons reported as bei...
Article
Full-text available
The ubiquitin-proteasome system (UPS) plays a fundamental role in protein degradation in neurons, and there is strong evidence that it fulfills a key role in synaptic transmission. The aim of the present work was to study the implication of one component of the UPS, the HERC1 E3 Ubiquitin Ligase, in motor function and neuromuscular transmission. Th...
Article
Full-text available
ABSTRACT Objective: To analyze the expression of transforming growth factor-ß1 in heterotopic grafts of adult dental apical papillae. Methodology: Adult apical papillae of Wistar rats were grafted in the ear of the same donor rats. 1, 3, 7 and 14 days after grafting, rats were perfused and the tissue containing the graft was processed for histologi...
Article
Full-text available
A mutation in the HERC2 gene has been linked to a severe neurodevelopmental disorder with similarities to the Angelman syndrome. This gene codifies a protein with ubiquitin ligase activity that regulates the activity of tumor protein p53 and is involved in important cellular processes such as DNA repair, cell cycle, cancer, and iron metabolism. Des...
Article
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Demyelinating disorders such as leukodystrophies and multiple sclerosis are neurodegenerative diseases characterized by the progressive loss of myelin that may lead toward a chronic demyelination of the brain's white matter, impairing normal axonal conduction velocity and ultimately causing neurodegeneration. Current treatments modifying the pathol...
Article
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The cerebellum plays a key role in the acquisition and execution of motor tasks whose physiological foundations were postulated on Purkinje cells' long-term depression (LTD). Numerous research efforts have been focused on understanding the cerebellum as a site of learning and/or memory storage. However, the controversy on which part of the cerebell...
Article
Naturally occurring neuronal death plays a substantial developmental role in the building of the neural circuitries. The neuronal death caused by different cerebellar mutations is mostly of an apoptotic nature. Apart from the identity of the intrinsic mechanisms of the mutations, adult cerebellar mutants are a powerful tool to causally study the de...
Article
Sympathoadrenal (SA) cell lineage encompasses neural crest derivatives such as sympathetic neurons, small intensely fluorescent (SIF) cells of sympathetic ganglia and adrenal medulla, and chromaffin cells of adrenal medulla and extra-adrenal paraganglia. SA autografts have been used for transplantation in Parkinson's disease (PD) for three reasons:...
Article
Apart from the pioneering studies of Ramon y Cajal [1893] and Rochon-Duvigneaud [1943], few studies have been devoted to the detailed study of the cytological and biochemical structure of the chameleon retina. In the present study we analyzed the expression of calbindin (CB), calretinin (CR) and parvalbumin (PV) immunoreactivities in the chameleon...
Article
Full-text available
The main goal of this study was the analysis of the developmental potentiality of tooth germ from late bell stage on, after its heterotopic placement within the skin. Teeth germs of newborn rats were grafted within a skin pouch of the ear of adult rats. Seven to fourteen days after grafting, dental germs developed normal dental structures in which...
Article
Full-text available
The main goal of this study was the analysis of the developmental potentiality of tooth germ from late bell stage on, after its heterotopic placement within the skin. Teeth germs of newborn rats were grafted within a skin pouch of the ear of adult rats. Seven to fourteen days after grafting, dental germs developed normal dental structures in which...
Chapter
The main goal of experimental therapies for the treatment of degenerative neurological diseases ranges from the prevention of molecular events underlying neuronal death to the replacement of concrete damaged neuronal populations. Invasive therapeutic strategies such as grafting of embryonic neural cells or of adult bone marrow stem cells, the induc...
Article
Antibodies directed against gamma-aminobutyric acid (GABA) and L-glutamic acid decarboxylases 65 and 67 kDa (GAD65 and -67) were used to study the GABAergic cell populations of the chameleon retina. GABA immunoreactivity was found in the two main types of retinal interneurons, amacrine and horizontal cells. Amacrine, displaced amacrine, and intra-...
Article
An tibodies d irected against gamma-aminobutyric (GABA) and L-glutamic acid decarboxylase (GAD) were u sed to study the GABAergic cell populations of the chameleon retina. GABA i m m u n o reactivity was found in the two main retinal in tern e u rons: amacrine and horizontal cells. Amacrine, displaced amacrine, intra- and i n t e r p l e x i f o rm...
Article
The distribution and ascending projections to the hypothalamic paraventricular nucleus of phenylethanolamine N-methyltransferase (PNMT)-immunoreactive perikaria were studied in adult pigeons using a combination of retrograde transport of Fluorogold injected into the paraventricular nucleus, and double immunohistochemical procedures for PNMT, tyrosi...
Article
1.The possibility that peripheral exogenous pyrogens can activate brainstem nuclei by abdominal viscera afferents was studied using the NADPH-diaphorase method in E. coli lipopolysaccharide treated rats with and without ibuprofen pre-treatment.2.NADPH-d staining revealed: (i) a significant increase in the number of NADPH-d labeled neurons in the su...
Article
We have tested the suitability of chromaffin-like carotid body glomus cells for dopamine cell replacement in Parkinsonian rats. Intrastriatal grafting of cell aggregates resulted in almost optimal abolishment of motor asymmetries and deficits of sensorimotor orientation. Recovery of transplanted animals was apparent 10 days after surgery and progre...
Article
Full-text available
This chapter summarizes some of the recent findings regarding the use of the eyelid motor system as a model for the study of reflex and learned motor responses and on the role played by cerebellar structures in the genesis and control of both types of movement. The eyelid motor system of the cat is used for the study of the kinetic properties of re...
Article
The eyelid motor system of the cat was used here for the study of the kinetic properties of reflex and conditioned lid movements, and of the role played by the cerebellum in the acquisition and/or performance of both types of motor responses. Spontaneous blinks, eyelid reflex responses, eye-guided lid movements and conditioned lid responses were re...
Article
This study determined whether macrophage inflammatory protein-1β (MIP-1β) plays a role in the hyperthermia caused by prostaglandin E2 (PGE2) given intracerebroventricularly (i.c.v.) in the rat. In these experiments, anti-murine MIP-1β antibody (anti-MIP-1β) was micro-injected in the anterior hypothalamic, preoptic area (AH/POA) just before i.c.v. P...
Article
Naturally occurring neuronal death was found by in situ labelling of nuclear DNA fragmentation during the development of the chick inferior olive. Counting neuronal perikarya showed an evident loss of cells from embryonic day 18 to hatching. This reduction in neuronal numbers was followed by an increase of similar size from days 1-4 post-hatching....
Article
Homotopic and isochronic transplantation of the alar plate of the rhombomeres 7 and 8 was performed between chick and quail embryos at the stage of 10-14 somites. Analysis of the graft derivatives in 12-day-old chimeric embryos by means of the quail nucleolar marker showed that the ipsilateral inferior olive is formed from the transplanted neuroepi...
Article
The use of horseradish peroxidase (HRP) and 1,1'-Dioctadecyl-3,3,3',3'- tetramethyl-indocarbocyanine perchlorate (DiI) as retrograde tracers, applied in vitro within the olivocerebellar tract of both embryos (9 to 21 days old) and postnatal (3-60 days old) chickens, has allowed the observation of a small population of neurons located ipsilaterally...
Article
Neuronal death of inferior olivary neurons after early axotomy of the olivocerebellar tract was studied in newborn (P1) hemicerebellectomized rats during the first six days after lesion. The degeneration of the inferior olive showed a topographic pattern from one (P2) to six days after axotomy (P7), after which this complex had almost completely di...
Article
Macrophage inflammatory protein-1 (MIP-1) evokes an intense fever, independent of a prostaglandin mechanism, and is now thought to play an important role in the defence response to bacterial pyrogens. The purpose of this study was 2-fold: (i) to determine whether the potent doublet of this cytokine, MIP-1beta, is actually produced in the brain in r...
Article
Abstract Macrophage inflammatory protein-1 (MIP-1) evokes an intense fever, independent of a prostaglandin mechanism, and is now thought to play an important role in the defence response to bacterial pyrogens. The purpose of this study was 2-fold: (i) to determine whether the potent doublet of this cytokine, MIP-1, is actually produced in the brai...
Article
Acridine Orange is commonly used as a fluorescent counterstain in fluorescent tract tracing techniques. Here we describe a method in which the substitution of the standard washing solutions (i.e., 0.9% saline) for a diluted solution of Acridine Orange (0.001%) during the perfusion of the animal before fixation provides a fluorescent counterstaining...
Article
The presence of ipsilateral inferior olivary neurons during postnatal development of the olivocerebellar projection in the rat was investigated by two in vitro axonal tracing methods and by the axotomy of one olivocerebellar tract. The experiments were carried out before (P1), during (P5-P10) and after (P20) the period of multiple innervation of Pu...
Article
In neonatal rats the injection of Fast Blue and Diamidino Yellow retrograde fluorescent tracers, each into separate cerebellar hemispheres, reveals the presence of double-labelled neurons positioned bilaterally in the inferior olivary complex during the early postnatal period (postnatal day 0 to postnatal day 5). This suggests that those neurons wh...
Article
Neuronal death in the inferior olivary complex (IOC) was studied in control and unilaterally pedunculotomized newborn rats, from postnatal day 1 (P1) to P30, in order to test whether the approximately two-fold increase in available specific targets (i.e. Purkinje cells) that is theoretically provided by sectioning one inferior cerebellar peduncle t...
Article
The early stages in the formation of Purkinje cell dendritic arbors have been analyzed using the horseradish peroxidase (HRP) ‘in vitro’ axonal tracing method, from embryonic day 19 (E19) to postnatal day 6 (P6). These stages comprise the transition from the bipolar Purkinje cell, at the end of its migration, to the phase of stellate cell with diso...
Article
Using three different retrograde tracing techniques, we analysed the broad distribution of ipsi-, contra- and bilateral NRTP neurons projecting to the cerebellum in the adult rat. The placement of the tracers into the cerebellar peduncles allowed us to determine the overall pattern of the NRTP-cerebellar projection. At the same time, the HRP "in vi...
Article
The membrane properties of the neurons located in the ventral part of the periaqueductal grey (PAG) of the guinea-pig were studied using an in vitro slice preparation. Cells had low values of resting membrane potential (-53.3 +/- 1.3 mV, mean +/- standard error), high input resistance (195. +/- 16.2 M ohm) and moderate values of membrane time const...
Article
This paper examines the organization of host afferents within cerebellar grafts implanted into kainic acid lesioned cerebellum. Our selection of a cerebellum, a prime example of a ‘point-to-point’ system, permits precise determination of the degree and the specificity of host-graft interactions. One month after a cerebellar injection of kainic acid...
Article
Using the Golgi method two types of cones were found in the chameleon retina: single and double cones, whose axons give a different pattern in the central and in the peripheral areas of the retinal. No rods were observed by this method in the retina of this animal.
Article
In this work we analyse the effect produced by reserpine on the development of thickness and cell number in the external granular layer in the cerebellum of chick embryo. A striking 48-hour histogenetic delay is observed in the treated embryos relative to controls, as show by greater thickness and cell density of this layer in the former, as well a...
Article
We describe the changes in the population of the macrophage cells during various phases of development of the anterior eye chamber and discuss the role these cells play in this developmental process in the chick.
Article
The present work describes the presence of some photoreceptor nuclei in the embryonic and adult chick retina which are situated outside of the external limiting membrane. We also discuss their possible degenerative character.
Article
The retina of the chick contains retinal cells of a morphology very similar to that of the horizontal cells, but the perikarya, axons, and axon terminals lie in the inner plexiform layer. The discovery of this neuronal ectopia appears to support the idea that some horizontal and amacrine cells derive from a common, freely migrating cell.
Article
Full-text available
The presence of dense core vesicles in the terminal expansions of photoreceptors in development is described in the chick embryo retina, from the 16th to the 18th day of incubation.
Article
The cones of the chameleon retina have, in the scleral portion of their inner segment, an oil drop whose morphology is similar in all the photoreceptors possessing this droplet. The irregularities in the membrane surrounding the lipid matrix show close relationships with the surrounding mitochondria.
Article
Full-text available
Among the types of horizontal cells of the avian retina, one has been described that has an axon terminating in a typical structure. The present study analyses the histogenesis of this axon whose initial outgrowth occurs on day 14 of incubation (HH-40). The axon terminal is first detectable, towards day 15 of incubation (HH-41), in the form of a va...
Article
We report a histogenetic study of the bipolar cells of the chick embryo retina between days 5 and 9, using the Golgi technique. On day 8, at the level of the developing outer plexiform layer, small delicate spines appear on the outer processes of the bipolar cells, which represents the commencement of their dendritic ramification. At a later staige...
Article
The cones of the chameleon retina have, in the scleral portion of their inner segment, an oil drop whose morphology is similar in all the photoreceptors possessing this droplet. The irregularities in the membrane surrounding the lipid matrix show close relationships with the surrounding mitochondria.Copyright © 1981 S. Karger AG, Basel
Article
Golgi's method shows that in the chick's retina 3 different types of horizontal cells can be detected. Two of the aforementioned have been analyzed previously; the 3rd type has not been previously described. It is a cell of intermediate size which is devoid of an axon.
Article
The present work deals with a comparative study between the morphology adopted by young retinal neuroblasts at their 10th and 11th day of incubation by the Golgi method, and the appearance of intensely autoradiographically marked nuclei in the inner nuclear, plexiform and ganglional layers. Likewise the same histogenetic origin is proposed for the...
Article
The present study provides us with some interesting data concering the morphology of neuroblastic differentiation in the tectum and the retina of an embryonic chicken subjected to a low dosage of gamma radiation on the fourth day of incubation. During this period the neuroblastic differentiation in the neuronal components still had not taken place,...
Article
In this study, the existence of intercellular junctions 'puncta adhaerentia' in the 12th day chick embryo retina, between cell processes of both outer and inner plexiform layers is shown. Coated and synaptic vesicles are also detected at this stage (HH-38) in bipolar, amacrine and photoreceptor cells. Synaptic vesicles are considered in this paper...

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