Abstract:
:Lysosomal storage diseases occur due to incomplete metabolic degradation of macromolecules by various hydrolytic enzymes in the lysosome. Despite structural differences, most of the lysosomal enzymes share many common features including a lysosomal targeting motif and phosphotransferase recognition sites. β-Glucuronidase (GUSB) is an important lysosomal enzyme involved in the degradation of glucuronate-containing glycosaminoglycan. The deficiency of GUSB causes mucopolysaccharidosis type VII (MPSVII), leading to lysosomal storage in the brain. GUSB is a well-studied protein for its expression, sequence, structure, and function. The purpose of this review is to summarize our current understanding of sequence, structure, function, and evolution of GUSB and its lysosomal enzyme targeting. Enzyme replacement therapy reported for this protein is also discussed.
journal_name
Rejuvenation Resjournal_title
Rejuvenation researchauthors
Naz H,Islam A,Waheed A,Sly WS,Ahmad F,Hassan Idoi
10.1089/rej.2013.1407subject
Has Abstractpub_date
2013-10-01 00:00:00pages
352-63issue
5eissn
1549-1684issn
1557-8577journal_volume
16pub_type
杂志文章,评审abstract::Previously, other groups and our team consistently have demonstrated that the possible origination of liver cancer stem cells (LCSCs) is the malignant transformation from liver normal stem cells (LNSCs). However, this complex and multi-step process is far from clear due to the accumulation of various gene dysregulatio...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2014.1642
更新日期:2015-08-01 00:00:00
abstract::Let us define human aging as the random accumulation of damage in the human organism's cells, tissues, or whole organism during life that increases the probability of death. This definition opens ways to postpone aging via risk factors to health. Studies show that changes in aging have postponed aging 0.15-0.20 years ...
journal_title:Rejuvenation research
pub_type: 杂志文章,评审
doi:10.1089/rej.2008.0675
更新日期:2008-04-01 00:00:00
abstract::Can memory be retained after cryopreservation? Our research has attempted to answer this long-standing question by using the nematode worm Caenorhabditis elegans, a well-known model organism for biological research that has generated revolutionary findings but has not been tested for memory retention after cryopreserv...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2014.1636
更新日期:2015-10-01 00:00:00
abstract::The progeroid Werner's syndrome (WS) represents the best current model of human aging. It is caused by loss of the WRN helicase/exonuclease, resulting in high levels of replication fork stalling and genomic instability. Current models suggest that characteristic WS phenotypes of poor S phase progression, low prolifera...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2006.0503
更新日期:2007-03-01 00:00:00
abstract::The notion that it is possible to eradicate age-related degeneration and live a life with a negligible rate of senescence solely by using a physical "repair-oriented" approach is flawed on a number of fronts. Here, I will argue that there are so many unknown variables embedded in this line of thinking that make the fi...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2014.1588
更新日期:2014-08-01 00:00:00
abstract::Osteoarthritis is the most common disabling condition of humans in the western world. It has been known for a very long time that aging is the most prominent risk factor for the initiation and progression of the disease, but the explanations for this phenomenon have changed over time. The most longstanding theory is t...
journal_title:Rejuvenation research
pub_type: 杂志文章,评审
doi:10.1089/1549168041552964
更新日期:2004-07-01 00:00:00
abstract::Theses reviewed in this issue include "Characterization and Purification of Putative Stem Cells from the Adult Murine Pancreas," "Inhibition of TLR4 Minimizes Islet Damage due to Sterile Inflammation and Improves Islet Transplant Outcomes," "Liquefaction of the Brain Following Stroke Shares Multiple Characteristics wi...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2020.2378
更新日期:2020-08-01 00:00:00
abstract:OBJECTIVE:This study examined the impact of estrogen replacement therapy with spirometry on pulmonary function in surgically castrated (salpingo-oophorectomy) postmenopausal women with genital prolapse. METHODS:The study included 60 postmenopausal women with pelvic organ prolapse. The study received institutional Ethi...
journal_title:Rejuvenation research
pub_type: 杂志文章,随机对照试验
doi:10.1089/rej.2012.1337
更新日期:2012-12-01 00:00:00
abstract::Mitochondria play a central role in cellular physiology. Besides their classic function of energy metabolism, mitochondria are involved in multiple cell functions, including energy distribution through the cell, energy/heat modulation, regulation of reactive oxygen species (ROS), calcium homeostasis, and control of ap...
journal_title:Rejuvenation research
pub_type: 杂志文章,评审
doi:10.1089/rej.2015.1704
更新日期:2016-02-01 00:00:00
abstract::Alzheimer's Disease (AD) is the most common age-related dementia, with a current prevalence in excess of five million individuals in the United States. The aggregation of amyloid-beta (A beta) into fibrillar amyloid plaques is a key pathological event in the development of the disease. Microglial proinflammatory activ...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2008.0757
更新日期:2008-10-01 00:00:00
abstract::Lysosomes are involved in degrading and recycling cellular ingredients, and their disruption with age may contribute to amyloidogenesis, paired helical filaments (PHFs), and α-synuclein and mutant huntingtin aggregation. Lysosomal cathepsins are upregulated by accumulating proteins and more so by the modulator Z-Phe-A...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2011.1282
更新日期:2012-04-01 00:00:00
abstract::d-galactose (d-gal) is widely used to induce aging. However, it is still unclear whether long-term injection of d-gal affects the gastrointestinal functions of aging rats, and how. In this study, we investigated the effects of d-gal on the gastrointestinal functions of aging rats, especially from the perspective of fe...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2020.2352
更新日期:2020-09-29 00:00:00
abstract::We examined the impact of strong co-presence and function of glutathione peroxidase-1 (GPX-1) and glial cell line-derived neurotrophic factor (GDNF) on protecting the rat dopaminergic pheochromocytoma cell line PC12 against 6-hydroxydopamine (6-OHDA) and hydrogen peroxide (H₂O₂) toxicities. Primarily, GPX-1 over-expre...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2012.1390
更新日期:2013-06-01 00:00:00
abstract::Treadmills are often used as rehabilitation devices to improve gait in Parkinson's disease (PD). Kinematic differences between treadmill and overground gait have been reported. However, electromyographic (EMG) patterns during treadmill and overground walking have not been systematically compared. The aim of this study...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2018.2084
更新日期:2019-02-01 00:00:00
abstract::Pluripotent stem cells offer unique opportunities for curing debilitating diseases. However, further comprehensive research is needed to better understand cell signaling during the differentiation of pluripotent cells into different cell lineages and accordingly to develop clinically applicable protocols. One of the l...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2011.1186
更新日期:2011-12-01 00:00:00
abstract::Aging is associated with protein damage and imbalance in redox status in a variety of cells and tissues, yet little is known about the extent of age-related oxidative stress in the peripheral nervous system. Previously, we showed a drastic decline in the expression of glial and neuronal proteins in myelinated peripher...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2009.0892
更新日期:2010-02-01 00:00:00
abstract::Telomeres are specialized DNA structures located at the ends of chromosomes. Their length is reduced at each cell cycle, especially when the cumulative burden of oxidative stress is high. The purpose of this study was to determine the associations between telomere length and clinical and biological risk factors in isc...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2011.1243
更新日期:2012-06-01 00:00:00
abstract::Intermittent fasting (IF) is an intervention that can be beneficial for health span and mitigate the risk of developing age-related cardiovascular diseases; however, the involved mechanisms are not well understood. The present study investigated the effects of IF regimens on the plasma level of angiotensin II (Ang II)...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2019.2254
更新日期:2020-08-01 00:00:00
abstract::Walnuts kernels (Juglans regia L.) have rich antioxidants content and have been used in both cosmetic and pharmaceutical industry. This study dealt with the protective role of walnut kernels extracts (WK) on isoproterenol (ISO)-induced myocardial infarction (MI) in rats. Rats were pretreated with WK extracts (300 mg/k...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2018.2140
更新日期:2019-08-01 00:00:00
abstract::This study was designed to elucidate the molecular mechanism underlying lysophosphatidic acid (LPA) and adenylyl cyclase inhibitor SQ22536 (ACI)-induced senescent human diploid fibroblast (HDF) proliferation. Because adenosine monophosphate (AMP)-activated protein kinase (AMPK) is known to inhibit cell proliferation, ...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2008.0709
更新日期:2008-08-01 00:00:00
abstract::There is a growing interest in the potential of mesenchymal stem cells (MSCs) for implementing regenerative medicine. We assessed the effect of intravenous administration of human bone marrow-derived MSC on the life span of a single Sprague-Dawley female rat. The treatment was started when the rat was 6 months old and...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2015.1777
更新日期:2016-08-01 00:00:00
abstract::Skeletal muscle fiber generation occurs principally in two myogenic phases: (1) Primary (embryonic) myogenesis when myoblasts proliferate and fuse to form primary myotubes and (2) secondary (fetal) myogenesis when successive waves of myoblasts fuse along the surface of the primary myotubes, giving rise to a population...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2010.1065
更新日期:2010-12-01 00:00:00
abstract::Spatial memory and cognition decline during aging. Montelukast, an FDA approved drug for the treatment of asthma, can restore spatial memory in old rats to levels similar to those of young animals. Treatment improves three hallmarks of aging in the brain: reducing microglial-mediated neuroinflammation, blood-brain bar...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2016.1903
更新日期:2016-12-01 00:00:00
abstract::Availability of reliable prognostic biomarkers that are also able to monitor preventive/therapeutic interventions in patients with mild cognitive impairment (MCI) is crucial. Cerebral brain-derived neurotrophic factor (BDNF) alterations were evidenced in Alzheimer's disease, but the value of blood BDNF in MCI is uncle...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2020.2307
更新日期:2020-10-01 00:00:00
abstract::Photoaging is the superposition of photodamage (ultraviolet [UV] radiation-induced) on the aging process. It is a major damaging consequence of free radical action. Curcumin (diferuloylmethane) is a phytochemical with diverse antioxidant and antiinflammatory properties. However, it shows a poor topical bioavailability...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2009.0906
更新日期:2010-08-01 00:00:00
abstract::The article is a presentation at the 4th Conference of ESAAM, which took place on October 30-31, 2015, in Athens, Greece. Its purpose was not to cover all aspects of cellular aging but to share with the audience of the Conference, in a 15-minute presentation, current knowledge about the rejuvenating and repairing soma...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2016.1809
更新日期:2017-02-01 00:00:00
abstract::Prunella vulgaris L., a well-known traditional Chinese herbal medicine, has anti-inflammatory and antioxidant activities. In the present study, the underlying molecular mechanisms of the protective effect of P. vulgaris extract (PVE) were investigated in UVB-irradiated normal human dermal fibroblasts (NHDFs). The mRNA...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2017.1971
更新日期:2018-08-01 00:00:00
abstract::Alzheimer's disease (AD) is a common form of dementia and amyloid-β peptide (Aβ) aggregation is considered to be one of its main causes. Paeoniflorin has been previously shown to attenuate cognitive damage inflicted by exogenous Aβ protein. Using transgenic Caenorhabditis elegans models expressing human Aβ1-42, we dem...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2017.1966
更新日期:2018-08-01 00:00:00
abstract:BACKGROUND:Curcumin, an extract from the rhizome of the plant Curcuma longa (turmeric), has been widely used as a spice and herbal medicine in Asia. It has been suggested to have many biological activities, such as antioxidative, antiinflammatory, anticancer, chemopreventive, and antineurodegenerative properties. We ev...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2010.1031
更新日期:2010-10-01 00:00:00
abstract::It is known that dietary restriction (DR) increases maximum longevity in rodents, but the mechanisms involved remain unknown. Among the possible mechanisms, several lines of evidence support the idea that decreases in mitochondrial oxidative stress and in insulin signaling are involved but it is not known if they are ...
journal_title:Rejuvenation research
pub_type: 杂志文章
doi:10.1089/rej.2008.0704
更新日期:2008-06-01 00:00:00