Abstract:
:Amyotrophic lateral sclerosis (ALS) is a complex neurodegenerative disease with clinical, pathological and genetic overlap with frontotemporal dementia (FTD). No longer viewed as one disease with a single unified cause, ALS is now considered to be a clinicopathological syndrome resulting from a complex convergence of genetic susceptibility, age-related loss of cellular homeostasis, and possible environmental influences. The rapid increase in recent years of the number of genes in which mutations have been associated with ALS has led to in vitro and in vivo models that have generated a wealth of data indicating disruption of specific biochemical pathways and sub-cellular compartments. Data implicating pathways including protein misfolding, mRNA splicing, oxidative stress, proteosome and mitochondrial dysfunction in the pathogenesis of ALS reinforce a disease model based on selective age-dependent vulnerability of a specific population of cells. To the clinical neurologist, however, ALS presents as a disease of focal onset and contiguous spread. Characteristic regional patterns of involvement and progression suggest that the disease does not proceed randomly but via a restricted number of anatomical pathways. These clinical observations combined with electrophysiological and brain-imaging studies underpin the concept of ALS at the macroscopic level as a 'system degeneration'. This dichotomy between cellular and systems neurobiology raises the fundamental questions of what initiates the disease process in a specific anatomical site and how the disease is propagated. Is the essence of ALS a cell-to-cell transmission of pathology with, for example, a 'prion-like' mechanism, or does the cellular pathology follow degeneration of specific synaptic networks? Elucidating the interaction between cellular degeneration and system level degeneration will aid modeling of the disease in the earliest phases, improve the development of sensitive markers of disease progression and response to therapy, and expand our understanding of the biological basis of clinical and pathological heterogeneity.
journal_name
J Anatjournal_title
Journal of anatomyauthors
Talbot Kdoi
10.1111/joa.12107subject
Has Abstractpub_date
2014-01-01 00:00:00pages
45-51issue
1eissn
0021-8782issn
1469-7580journal_volume
224pub_type
杂志文章,评审abstract::A histological study has shown that considerable amounts of lipid accumulate in the oxyntico-peptic cells of the proventriculus of domestic fowls deprived of food. Lipid droplets were first seen in the cells 6 hours after commencing the fast and they reached a maximum in 24 hours; a decrease was detected histologicall...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:
更新日期:1975-12-01 00:00:00
abstract::There is need for a consistent definition of structures caudal to the testis that variously are termed 'gubernaculum testis' as a basis for understanding the emergence and sexually dimorphic further growth and differentiation of this specifically mammalian structure. Rodent fetuses undergo a stage of development durin...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:
更新日期:1996-08-01 00:00:00
abstract::The ultrastructure of the tammar placenta was studied throughout pregnancy. The uterine epithelium grows from a columnar to an enlarged, undulating epithelium between early gestation and mid-gestation when the shell coat that surrounds the marsupial conceptus ruptures. Trophectoderm and uterine epithelium do not form ...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:10.1046/j.1469-7580.2002.00084.x
更新日期:2002-08-01 00:00:00
abstract::The morphology of the cartilage-marrow interface in chick embryo tibiae has been studied from Day 11 to Day 14. The cartilage-marrow interface did not present a uniform aspect and three different areas could be defined. Most of the interface was lined by fibroblast-like cells, macrophage-like cells and multinucleate g...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:
更新日期:1988-10-01 00:00:00
abstract::Angiogenesis is essential for the replacement of cartilage by bone during growth and repair. In order to obtain a better understanding of the mechanisms regulating vascular invasion at sites of endochondral ossification we have investigated the expression of the endothelial cell-specific mitogen, vascular endothelial ...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:10.1046/j.1469-7580.1999.19440519.x
更新日期:1999-05-01 00:00:00
abstract::Kinetic changes have been studied in the transitional cells of the marrow in normal, hypoxic (0.5 atm) and rebound rats. Suspensions of marrow cells were incubated with tritiated thymidine for 1 hour, and the percentage of labelled transitionals of all sizes, as well as of large transitionals, was couinted in radioaut...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:
更新日期:1980-03-01 00:00:00
abstract::Mammalian neonates are born at a wide range of maturity levels. Altricial newborns are born with limited sensory agency and require extensive parental care. In contrast, precocial neonates are relatively mature physically and often capable of independent function shortly after birth. In extant mammals, placental newbo...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:10.1111/joa.13127
更新日期:2020-04-01 00:00:00
abstract::A study of the attachments of the musculotendinous fibres of the lumbar erector spinae muscle shows that it is made of two parts: a superficial or costal part, and a deep or vertebral part. The superficial part arises by small fleshy slips from the posterior (superficial) surface as well as the rostral and lateral mar...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:
更新日期:1986-02-01 00:00:00
abstract::Degeneration induced by intracranial section of the ophthalmic branch of the trigeminal nerve was used as a marker to trace the ocular passage and termination of fibres in ocular filtration angle structures of 3 cynomolgus monkeys. Fine supraciliary nerves branched from precorneal nerves as they entered the sclera fro...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:
更新日期:1994-06-01 00:00:00
abstract::Knowledge of the pattern of human craniofacial development in the fetal period is important for understanding the mechanisms underlying the emergence of variations in human craniofacial morphology. However, the precise character of the prenatal ontogenetic development of the human cranium has yet to be fully establish...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:10.1111/j.1469-7580.2008.00884.x
更新日期:2008-05-01 00:00:00
abstract::Fresh specimens of human peritoneum collected from heart-beating cadaver organ donors have been examined by transmission electron microscopy. Samples were taken from the anterior abdominal wall and from the surfaces of the liver, stomach and diaphragm. The mesothelium consisted of a single layer of flattened cells gen...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:
更新日期:1989-12-01 00:00:00
abstract::The effects of a chronic administration of angiotensin II on the zona fasciculata of rats treated with dexamethasone and maintenance doses of ACTH were investigated by morphometric methods applied to electron microscopy. It was found that angiotensin induced a significant increase in the volume of cells, nuclei, mitoc...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:
更新日期:1981-03-01 00:00:00
abstract::The pulmonary intravascular macrophages (PIMs) of domestic ungulates are recognised by their specific surface coat, consisting of linearly arranged globules along the external leaf of the plasma membrane. The coat is sensitive to in vitro digestion with lipolytic lipase (LPL), intravenous heparin and clinical exposure...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:10.1046/j.1469-7580.2001.19850611.x
更新日期:2001-05-01 00:00:00
abstract::The effects of excess iodine on the development of the thyroid gland of chick embryos was assessed following injections of potassium iodide prior to incubation. Iodide injection resulted in a significantly greater thyroid gland weight (goitre) on Day 18 of incubation and a delay in hatching. Histological studies of th...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:
更新日期:1991-06-01 00:00:00
abstract::Four forelimbs of 3 platypuses and 3 forelimbs of 2 echidnas were examined to study the precise form of the brachial plexus and to clarify the structural characteristics of the brachial plexus in phylogeny. The spinal components contributing to the plexus (C4-T2) and the formation patterns of the 3 trunks of the plexu...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:10.1046/j.1469-7580.1997.19030447.x
更新日期:1997-04-01 00:00:00
abstract::The European eel (Anguilla anguilla) has been extensively studied, especially because of its highly specialized migratory behaviour associated with substantial phenotypic transformations. During this migration, one of those transformations the eel undergoes is from yellow to silver eel, a process known as silvering. A...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:10.1111/joa.13259
更新日期:2020-11-01 00:00:00
abstract::A one day symposium of the Anatomical Society of Great Britain and Ireland was held at the Royal Free Hospital in London on the 17th September 1999, on the topic of 'Mechanisms of ageing and longevity'. The following are abstracts of communications and posters presented at the meeting. ...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:10.1046/j.1469-7580.2000.19620293.x
更新日期:2000-02-01 00:00:00
abstract::The production of echolocation calls in bats along with forces produced by contraction of thoracic musculature used in flight presumably puts relatively high mechanical loads on the lower respiratory tract (LRT). Thus, there are likely adaptations to prevent collapse or distortion of the bronchial tree and trachea dur...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:10.1111/joa.12575
更新日期:2017-04-01 00:00:00
abstract::The ligamentous attachments of the lumbar spinous processes were studied by dissection and histological examination in seven dogs, three cats and six baboons. Unlike man, the lumbar spines of these animals do not possess a supraspinous ligament and there is no decussation of the erector spinae tendons in the lower lum...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:
更新日期:1980-03-01 00:00:00
abstract::The fine structure of the elastic cartilage of the pinna has been examined in young rabbits aged from 1 day to 1108 days. Changes associated with growth and development are related not only to age but also to the actual situation in the pinna. In the midline, progressive changes are seen from the tip to the base. The ...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:
更新日期:1977-04-01 00:00:00
abstract::Retrograde fluorescent transport of Fast Blue (FB) and Diamidino Yellow (DY) was used to study the localisation of neurons that innervate the palpebral conjunctiva and the superior tarsal muscle in the cynomolgous monkey. Labelled cell bodies of sensory neurons including a few double labelled cell bodies were found in...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:
更新日期:1996-10-01 00:00:00
abstract::The knee joint consists of multiple interacting tissues that are prone to injury- and disease-related degeneration. Although much is known about the structure and function of the knee's constituent tissues, relatively little is known about their cellular origin and the mechanisms governing their segregation. To invest...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:10.1111/j.1469-7580.2008.00966.x
更新日期:2008-11-01 00:00:00
abstract::The proportion of total limb length taken up by the individual skeletal elements (limb proportionality), varies widely between species. These diverse skeletal forms have evolved to allow for a range of limb uses and they first emerge as the embryo develops, to achieve the characteristic skeletal architecture of each s...
journal_title:Journal of anatomy
pub_type: 杂志文章,评审
doi:10.1111/joa.12171
更新日期:2014-06-01 00:00:00
abstract::Collagen fibrils from different rat tendons have been investigated by freeze-fracture and transmission electron microscopy. In all cases, marked differences in both fibril morphology and subfibrillar organisation have been consistently found between the tendon core (composed of large and heterogeneous fibrils comprisi...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:
更新日期:1990-10-01 00:00:00
abstract::Muscles are considered to play an important role in the ongoing daily loading of bone, especially in the masticatory apparatus. Currently, there are no measurements describing this role over longer periods of time. We made simultaneous and wireless in vivo recordings of habitual strains of the rabbit mandible and mass...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:10.1111/j.1469-7580.2011.01375.x
更新日期:2011-06-01 00:00:00
abstract::A combination of stereology and physiology is used to estimate the morphometric diffusing capacity for oxygen of the normal human placenta at term. The morphometric diffusing capacity is found to be higher than published values determined by physiological methods. The most likely explanation for this discrepancy is th...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:
更新日期:1984-12-01 00:00:00
abstract::It has been shown that gibbons are able to brachiate with very low mechanical costs. The conversion of muscle activity into smooth, purposeful movement of the limb depends on the morphometry of muscles and their mechanical action on the skeleton. Despite the gibbon's reputation for excellence in brachiation, little in...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:10.1111/j.1469-7580.2009.01109.x
更新日期:2009-09-01 00:00:00
abstract::The blood supply of the symphysis pubis is still the subject of some debate. Classic anatomy books state that this joint is avascular, whereas some published works have shown blood vessels in young specimens. As several articular discs such as the knee menisci are known to have blood vessels in their peripheries, we d...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:10.1111/j.0021-8782.2004.00271.x
更新日期:2004-03-01 00:00:00
abstract::This study addresses the effects of increased mechanical load on the vertebral bone of post-smolt Atlantic salmon by forcing them to swim at controlled speeds. The fish swam continuously in four circular tanks for 9 weeks, two groups at 0.47 body lengths (bl) × s(-1) (non-exercised group) and two groups at 2 bl × s(-1...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:10.1111/j.1469-7580.2011.01399.x
更新日期:2011-10-01 00:00:00
abstract::We investigated the occurrence and anatomy of the vomeronasal system (VNS) in tadpoles of 13 different anuran species. All of the species possessed a morphologically fully developed VNS with a highly conserved anatomical organisation. We found that a bean-shaped vomeronasal organ (VNO) developed early in the tadpoles,...
journal_title:Journal of anatomy
pub_type: 杂志文章
doi:10.1111/j.1469-7580.2012.01543.x
更新日期:2012-10-01 00:00:00