scholarly journals The Pathological Features of Common Hereditary Mitochondrial Dynamics Neuropathy

2021 ◽  
Vol 15 ◽  
Author(s):  
Rui Wu ◽  
He Lv ◽  
Hui Wang ◽  
Zhaoxia Wang ◽  
Yun Yuan
Keyword(s):  
Morphological Changes ◽  
Mitochondrial Dynamics ◽  
Deep Tendon Reflex ◽  
Nerve Fibers ◽  
Sensory Loss ◽  
Mitofusin 2 ◽  
Charcot Marie Tooth Disease ◽  
Tendon Reflex ◽  
Unmyelinated Nerve ◽  
Related Proteins

ObjectivesMitofusin 2 and ganglioside-induced differentiation-associated protein 1 are two main mitochondrial dynamics-related proteins. Dysfunction of these two proteins leads to different subtypes of Charcot–Marie–Tooth disease type 2A (CMT2A) and CMT2K. This study aims to report the pathological difference between CMT2A and CMT2K in a large cohort.MethodsThirty patients with molecularly confirmed CMT2A and nine with CMT2K were identified by next-generation sequencing. Sural nerve biopsies were performed in 29 patients.ResultsThe patients with both diseases showed length-dependent neuropathy with distal weakness, sensory loss, and no deep tendon reflex. Optic neuropathy appeared in 3/30 (10%) patients with CMT2A. Tendon contracture appeared in 4/9 (50.0%) patients with CMT2K. Sural biopsy revealed the loss of both myelinated and unmyelinated nerve fibers. Closely packed, irregularly oriented neurofilaments were observed in axons of unmyelinated nerve fibers in both diseases. Another important finding was the ubiquitous presence of smaller, rounded, and fragmented mitochondria in CMT2A and elongated mitochondria in CMT2K in the myelinated and unmyelinated axons.ConclusionThis study confirmed large diversity in phenotypes between CMT2A and CMT2K. Mitochondrial dynamics-related variations can induce different mitochondrial morphological changes and neurofilament accumulation in axons.

scholarly journals A study on peripheral neuropathy in patients with diabetic foot ulcers

2018 ◽  
Vol 5 (3) ◽  
pp. 913
Author(s):  
Siddesh Kumar M. H. ◽  
Moosabba M. S. ◽  
Sanjay N. Koppad
Keyword(s):  
Peripheral Neuropathy ◽  
Diabetic Foot ◽  
Deep Tendon Reflex ◽  
Nerve Fibers ◽  
Diabetic Neuropathies ◽  
Sensory Loss ◽  
Foot Ulcers ◽  
Diabetic Foot Ulcers ◽  
Men And Women ◽  
Tendon Reflex

Background: Diabetic neuropathies are nerve disorders associated with diabetes. The most common complication of diabetes is caused by hyperglycemia which can damage nerve fibers throughout the body. Depending on the types of nerves involved, diabetic neuropathies can be categorized as peripheral, autonomic, proximal, focal neuropathies.Methods: A total of 62 diabetic foot patients admitted in general surgery department of Yenepoya medical college and hospital undergo neurological examination. Patients who were having peripheral neuropathy with diabetic foot ulcer between 18 and 85 years of age were included in the study.Results: On 62 patients with diabetic foot ulcers, 50 were unilateral and 12 were bilateral among which 8 patients had undergone toe amputation prior to examination. Patients were predominantly male 48 (77.4%). There were 14 women and 48 men with an average duration of diabetics being 15.6 and 14.8 years respectively. Women   were older than male patient (58.2 v/s 51.7 years). Study of motor and sensory signs men and women patients, abnormal deep tendon reflex and deep sensory loss was high. Abnormal deep tendon reflex was 38 (79.1%) and 11 (78.5%), atrophy was 24 (50%) and 2 (14.2%), loss of pain was 18 (37%) and 4 (28.5%), loss of touch was 32 (66%) and 8 (57%), and deep sensory loss was 42 (87.5%) and 12 (85.7%) in men and women respectively.Conclusions: Diabetes mellitus leads to neuropathies of more than one type and all contribute to diabetic foot pathogenesis. Clinical symptoms and signs, as well as nerve conduction studies may be different between men and women with diabetic foot. Motor neuropathies may constitute an important prognostic parameter in men. Mononeuropathies sometimes reflect more severe involvement and peroneal and ulnar neuropathy is remarkable among these.

scholarly journals Astragaloside II Ameliorated Podocyte Injury and Mitochondrial Dysfunction in Streptozotocin-Induced Diabetic Rats

2021 ◽  
Vol 12 ◽  
Author(s):  
Jun Su ◽  
Chongting Gao ◽  
Ling Xie ◽  
Ying Fan ◽  
Yilan Shen ◽  
...  
Keyword(s):  
Mitochondrial Dysfunction ◽  
Tissue Injury ◽  
Morphological Changes ◽  
Mitochondrial Dynamics ◽  
Diabetic Rats ◽  
Control Group ◽  
Protective Effects ◽  
Podocyte Injury ◽  
Renal Histopathology ◽  
Related Proteins

Astragaloside II (AS II), a novel saponin purified from Astragalus membranes, has been reported to modulate the immune response, repair tissue injury, and prevent inflammatory response. However, the protective effects of AS II on podocyte injury in diabetic nephropathy (DN) have not been investigated yet. In this study, we aimed to investigate the beneficial effects of AS II on podocyte injury and mitochondrial dysfunction in DN. Diabetes was induced with streptozotocin (STZ) by intraperitoneal injection at 55 mg/kg in rats. Diabetic rats were randomly divided into four groups, namely, diabetic rats and diabetic rats treated with losartan (10 mg·kg−1·d−1) or AS II (3.2 and 6.4 mg·kg−1·d−1) for 9 weeks. Normal Sprague-Dawley rats were chosen as nondiabetic control group. Urinary albumin/creatinine ratio (ACR), biochemical parameters, renal histopathology and podocyte apoptosis, and morphological changes were evaluated. Expressions of mitochondrial dynamics-related and autophagy-related proteins, such as Mfn2, Fis1, P62, and LC3, as well as Nrf2, Keap1, PINK1, and Parkin, were examined by immunohistochemistry, western blot, and real-time PCR, respectively. Our results indicated that AS II ameliorated albuminuria, renal histopathology, and podocyte foot process effacement and podocyte apoptosis in diabetic rats. AS II also partially restored the renal expression of mitochondrial dynamics-related and autophagy-related proteins, including Mfn2, Fis1, P62, and LC3. AS II also increased the expression of PINK1 and Parkin associated with mitophagy in diabetic rats. Moreover, AS II facilitated antioxidative stress ability via increasing Nrf2 expression and decreasing Keap1 protein level. These results suggested that AS II ameliorated podocyte injury and mitochondrial dysfunction in diabetic rats partly through regulation of Nrf2 and PINK1 pathway. These important findings might provide an innovative therapeutic strategy for the treatment of DN.

Dermal nerves in monzygotic twins discordant for diabetes: Morphometric study of perineurial cell basement membrane

1987 ◽  
Vol 45 ◽  
pp. 844-845
Author(s):  
J. L. Beggs ◽  
P. C. Johnson ◽  
C. J. Watkins ◽  
A. G. Olafsen ◽  
C. P. Jones
Keyword(s):  
Basement Membrane ◽  
Morphological Changes ◽  
Monozygotic Twins ◽  
Nerve Fibers ◽  
Morphometric Study ◽  
Perineurial Cell ◽  
Membrane Morphology ◽  
Cell Layers ◽  
Unmyelinated Nerve ◽  
Perineurial Sheath

Nerves in the dermal layer of skin are typically composed of several myelinated and unmyelinated nerve fibers surrounded by a perineurial sheath. Generally, this sheath is composed of one to two perineurial cell layers. The perineurial sheath helps to maintain a suitable environment for proper nerve function (1) and morphological changes in the sheath may cause nerve dysfunction (2). Perineurial cell basement membrane (PCBM) becomes thickened in diabetics and this phenomenon has been viewed as a diagnostic aid in the detection of diabetes (3). Since other factors (e.g. genetics, aging, vascular disease) may play a role in basement membrane morphology, we undertook this study of dermal nerves from identical twins discordant for diabetes to determine the effects of diabetic dysmetabolism on morphological changes in the PCBM. Since monozygotic twins have an identical genetic composition, it is likely that any significant differences in the dermal nerve would be due to factors related to diabetes.

scholarly journals Mechanical Sensing Element PDLIM5 Promotes Osteogenesis of Human Fibroblasts by Affecting the Activity of Microfilaments

Biomolecules ◽  
2021 ◽  
Vol 11 (5) ◽  
pp. 759
Author(s):  
Xiaolan Huang ◽  
Rongmei Qu ◽  
Yan Peng ◽  
Yuchao Yang ◽  
Tingyu Fan ◽  
...  
Keyword(s):  
Osteogenic Differentiation ◽  
Morphological Changes ◽  
Human Fibroblasts ◽  
Inhibitory Effect ◽  
Induction Medium ◽  
Sensing Element ◽  
Differentiation Potential ◽  
Osteogenic Induction Medium ◽  
Osteogenic Induction ◽  
Related Proteins

Human skin fibroblasts (HSFs) approximate the multidirectional differentiation potential of mesenchymal stem cells, so they are often used in differentiation, cell cultures, and injury repair. They are an important seed source in the field of bone tissue engineering. However, there are a few studies describing the mechanism of osteogenic differentiation of HSFs. Here, osteogenic induction medium was used to induce fibroblasts to differentiate into osteoblasts, and the role of the mechanical sensitive element PDLIM5 in microfilament-mediated osteogenic differentiation of human fibroblasts was evaluated. The depolymerization of microfilaments inhibited the expression of osteogenesis-related proteins and alkaline phosphatase activity of HSFs, while the polymerization of microfilaments enhanced the osteogenic differentiation of HSFs. The evaluation of potential protein molecules affecting changes in microfilaments showed that during the osteogenic differentiation of HSFs, the expression of PDLIM5 increased with increasing induction time, and decreased under the state of microfilament depolymerization. Lentivirus-mediated PDLIM5 knockdown by shRNA weakened the osteogenic differentiation ability of HSFs and inhibited the expression and morphological changes of microfilament protein. The inhibitory effect of knocking down PDLIM5 on HSF osteogenic differentiation was reversed by a microfilament stabilizer. Taken together, these data suggest that PDLIM5 can mediate the osteogenic differentiation of fibroblasts by affecting the formation and polymerization of microfilaments.

TENDON REFLEX AND STRATEGIES FOR QUANTIFICATION, WITH NOVEL METHODS INCORPORATING WIRELESS ACCELEROMETER REFLEX QUANTIFICATION DEVICES, A PERSPECTIVE REVIEW

2011 ◽  
Vol 11 (03) ◽  
pp. 471-513 ◽  
Author(s):  
ROBERT LEMOYNE ◽  
TIMOTHY MASTROIANNI ◽  
CRISTIAN COROIAN ◽  
WARREN GRUNDFEST
Keyword(s):  
Longitudinal Study ◽  
Patellar Tendon ◽  
Neurological Examination ◽  
Deep Tendon Reflex ◽  
Reflex Response ◽  
Fundamental Aspect ◽  
Tendon Reflex ◽  
Reflex System ◽  
Wireless Accelerometer ◽  
Tendon Reflexes

The deep tendon reflex is a fundamental aspect of a neurological examination. The two major parameters of the tendon reflex are response and latency, which are presently evaluated qualitatively during a neurological examination. The reflex loop is capable of providing insight into the status and therapy response of both upper and lower motor neuron syndromes. Attempts have been made to ascertain reflex response and latency; however, these systems are relatively complex, resource intensive, with issues of consistent and reliable accuracy. The solution presented is a wireless quantified reflex device using tandem three-dimensional (3D) wireless accelerometers to obtain response based on acceleration waveform amplitude and latency derived from temporal acceleration waveform disparity. Three specific aims have been established for the proposed wireless quantified reflex device: (1) Demonstrate the wireless quantified reflex device is reliably capable of ascertaining quantified reflex response and latency using a quantified input. (2) Evaluate the precision of the device using an artificial reflex system. (3) Conduct a longitudinal study respective of subjects with healthy patellar tendon reflexes, using the wireless quantified reflex evaluation device to obtain quantified reflex response and latency. Aim 1 has led to a steady evolution of the wireless quantified reflex device from a singular 2D wireless accelerometer capable of measuring reflex response to a tandem 3D wireless accelerometer capable of reliably measuring reflex response and latency. The hypothesis for aim 1 is that a reflex quantification device can be established for reliably measuring reflex response and latency for the patellar tendon reflex, comprised of an integrated system of wireless 3D MEMS accelerometers. Aim 2 further emphasized the reliability of the wireless quantified reflex device by evaluating an artificial reflex system. The hypothesis for aim 2 is that the wireless quantified reflex device can obtain reliable reflex parameters (response and latency) from an artificial reflex device. Aim 3 synthesizes the findings relevant to aim 1 and 2, while applying the wireless accelerometer reflex quantification device to a longitudinal study of healthy patellar tendon reflexes. The hypothesis for aim 3 is that during a longitudinal evaluation of the deep tendon reflex the parameters for reflex response and latency can be measured with a considerable degree of accuracy, reliability, and reproducibility. Enclosed is a detailed description of a wireless quantified reflex device with research findings and potential utility of the system, inclusive of a comprehensive description of tendon reflexes, prior reflex quantification systems, and correlated applications.

scholarly journals Effects of Millimolar Steady-State Hydrogen Peroxide Exposure on Inflammatory and Redox Gene Expression in Immune Cells from Humans with Metabolic Syndrome

Nutrients ◽  
2018 ◽  
Vol 10 (12) ◽  
pp. 1920 ◽  
Author(s):  
Carla Busquets-Cortés ◽  
Xavier Capó ◽  
Emma Argelich ◽  
Miguel Ferrer ◽  
David Mateos ◽  
...  
Keyword(s):  
Gene Expression ◽  
Metabolic Syndrome ◽  
Hydrogen Peroxide ◽  
Immune Cells ◽  
Mononuclear Cells ◽  
Ex Vivo ◽  
Mitochondrial Dynamics ◽  
High Concentration ◽  
Anti Inflammatory ◽  
Related Proteins

Reactive oxygen species (ROS) such as hydrogen peroxide (H2O2) can exert opposed effects depending on the dosage: low levels can be involved in signalling and adaptive processes, while higher levels can exert deleterious effects in cells and tissues. Our aim was to emulate a chronic ex vivo oxidative stress situation through a 2 h exposure of immune cells to sustained H2O2 produced by glucose oxidase (GOX), at high or low production rate, in order to determine dissimilar responses of peripheral blood mononuclear cells (PBMCs) and neutrophils on ROS and cytokine production, and mitochondrial dynamics-related proteins, pro/anti-inflammatory and anti-oxidant gene expression. Immune cells were obtained from subjects with metabolic syndrome. H2O2 at low concentrations can trigger a transient anti-inflammatory adiponectin secretion and reduced gene expression of toll-like receptors (TLRs) in PBMCs but may act as a stimulator of proinflammatory genes (IL6, IL8) and mitochondrial dynamics-related proteins (Mtf2, NRF2, Tfam). H2O2 at a high concentration enhances the expression of pro-inflammatory genes (TLR2 and IL1β) and diminishes the expression of mitochondrial dynamics-related proteins (Mtf1, Tfam) and antioxidant enzymes (Cu/Zn SOD) in PBMCs. The GOX treatments produce dissimilar changes in immune cells: Neutrophils were more resistant to H2O2 effects and exhibited a more constant response in terms of gene expression than PBMCs. We observe emerging roles of H2O2 in mitochondrial dynamics and redox and inflammation processes in immune cells.

Deep Tendon Reflex in Eaton-Lambert Syndrome

1978 ◽  
Vol 32 (1) ◽  
pp. 109-113
Author(s):  
Hitoka Doi ◽  
Yoshiyuki Murai ◽  
Yoshigoro Kuroiwa
Keyword(s):  
Deep Tendon Reflex ◽  
Tendon Reflex

QUANTIFIED DEEP TENDON REFLEX DEVICE, SECOND GENERATION

2008 ◽  
Vol 08 (01) ◽  
pp. 75-85 ◽  
Author(s):  
ROBERT LEMOYNE ◽  
FOAD DABIRI ◽  
ROOZBEH JAFARI
Keyword(s):  
Traumatic Brain Injury ◽  
Brain Injury ◽  
Stretch Reflex ◽  
Evaluation System ◽  
Deep Tendon Reflex ◽  
Reflex Response ◽  
Fundamental Aspect ◽  
Maximum Acceleration ◽  
Mems Accelerometer ◽  
Tendon Reflex

The deep tendon reflex is a fundamental aspect of neurological examinations. The severity of and degree of recovery from a traumatic brain injury can be assessed by the myotatic stretch reflex. A hyperactive reflex response is correlated with spasticity, which can also be correlated with the degree of damage to the supraspinal input, in essence assessing the severity of traumatic brain injury. The myotatic stretch reflex is clinically evaluated by the National Institute of Neurological Disorders and Stroke (NINDS) reflex scale (0–4); however, this scale lacks temporal data and may also vary in interpretation. The solution is a fully quantified evaluation system of the myotatic stretch reflex, whereby a patellar hammer's force input is based on original potential energy and a microelectromechanical system (MEMS) accelerometer quantifies the output. The MEMS accelerometer is attached to a set anchor point near the ankle. The reflex amplitude is based on the maximum acceleration of the reflex response. The quantified data collected from MEMS accelerometers are transmitted by a portable computer (i.e. a Pocket PC). This paper describes a device that quantitatively evaluates the reflex response using accelerometers and that demonstrates precision for reproducibility.

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