Abstract:
:Huntington's disease (HD) is a devastating neurodegenerative disorder that occurs in patients with a mutation in the huntingtin or IT15 gene. Patients are plagued by early cognitive signs, motor deficits, and psychiatric disturbances. Symptoms are attributed to cell death in the striatum and disruption of cortical-striatal circuitry. Mechanisms of cell death are unclear, but processes involving mitochondrial abnormalities, excitotoxicity, and abnormal protein degradation have been implicated. Many factors likely contribute to neuron death and dysfunction, and this has made it difficult to systematically address the pathology in HD. Pharmaceutical therapies are commonly used in patients to treat disease symptoms. These have limited benefit and do not address the inexorable disease progression. Several neuroprotective therapies are being evaluated in animal models of HD as well as in clinical trials. Similarly, cell replacement strategies such as fetal transplantation have been used in the clinic with minimal success, making future cell replacement strategies such as stem cell therapy uncertain. This review describes the disease pathology in HD and addresses many of the past and emerging therapeutic strategies.
journal_name
Cell Transplantjournal_title
Cell transplantationauthors
Ramaswamy S,Shannon KM,Kordower JHdoi
10.3727/000000007783464687subject
Has Abstractpub_date
2007-01-01 00:00:00pages
301-12issue
3eissn
0963-6897issn
1555-3892journal_volume
16pub_type
杂志文章,评审abstract::The incidence of cell transplant-transmitted infection is unknown and can only be inferred from prospective studies--that have not yet been performed and reported. The possibility of donor-to-recipient disease transmission through cell transplant therapy can be considered by reviewing the risk associated with other tr...
journal_title:Cell transplantation
pub_type: 杂志文章,评审
doi:10.1016/0963-6897(95)00035-v
更新日期:1995-09-01 00:00:00
abstract::Human embryonic stem (hES) cells have the ability to differentiate into a variety of different cell lineages and potentially provide a source of differentiated cells for many therapeutic uses. Here we investigated an efficient method of hepatic differentiation from hES cells. A human ES cell line, KhES-1, was used and...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/000000006783981305
更新日期:2006-01-01 00:00:00
abstract::Previously, we found that hepatocyte growth factor receptor (c-Met)-and alpha-fetoprotein (AFP)-expressing cells were present in adult rat bone marrow, and that these cells also expressed hematopoietic stem cell markers, such as CD34, Thy-1, and c-Kit. When bone marrow cells were cultured in a hepatocyte growth medium...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/000000004783983800
更新日期:2004-01-01 00:00:00
abstract::The major limitation of nonhuman primate (NHP) embryonic stem (ES) cell research is inefficient genetic modification and limited knowledge of differentiation mechanisms. A genetically modified NHP-ES cell with biomarkers, such as green fluorescent protein (GFP), that allow noninvasive monitoring of transgenic cells, i...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/000000002783985350
更新日期:2002-01-01 00:00:00
abstract::We investigated the effects of transplantation of CXCR4-overexpressing adipose tissue-derived stem cells (ADSCs) into a mouse diabetic hindlimb ischemia model on homing and engraftment as early as 48 h after transplant. CXCR4-overexpressing ADSCs were intramuscularly or intravenously injected into diabetic mice with h...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/096368916X692708
更新日期:2017-02-16 00:00:00
abstract::Pancreatic beta cell lines may offer an abundant source of cells for beta-cell replacement in type I diabetes. Using regulatory elements of the bacterial tetracycline (tet) operon for conditional expression of SV40 T antigen oncoprotein in transgenic mouse beta cells, we have shown that reversible immortalization is a...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:
更新日期:2001-01-01 00:00:00
abstract::To examine the effects of autologous sural nerve and adrenal medullary tissue intrastriatal cografts upon voluntary motor performance in parkinsonism, a non-human primate 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) model was employed to quantitatively assess skilled hand movements. Motor performance was studie...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.1016/0963-6897(95)92155-r
更新日期:1995-01-01 00:00:00
abstract::ET-Kyoto solution (ET-K) is an extracellular-type organ preservation solution containing the cytoprotective disaccharide, trehalose. A previous study reported the supplement of dibutyryl cyclic adenosine monophosphate (db-cAMP) in conventional ET-K to attenuate lung ischemia-reperfusion injury. In this study, the effi...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/000000008783906928
更新日期:2008-01-01 00:00:00
abstract::In our previous study, intracerebral implantation of peripheral blood stem cells (PBSCs) improved functional outcome in rats with chronic cerebral infarction. Based on this finding, a randomized, single blind controlled study was conducted in 30 patients [PBSC group (n = 15) and control group (n = 15)] with middle cer...
journal_title:Cell transplantation
pub_type: 杂志文章,随机对照试验
doi:10.3727/096368914X678562
更新日期:2014-01-01 00:00:00
abstract::Hepatocyte transplantation is dependent on the availability of good quality human hepatocytes isolated from donor liver tissue. Hepatocytes obtained from livers rejected for transplantation on the grounds of steatosis are often of low viability and not suitable for clinical use. The aim of this study was to evaluate t...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/096368910X514620
更新日期:2010-01-01 00:00:00
abstract::Prior studies have suggested the possibility of immune-mediated death of xenogeneic hepatocytes in a bioartificial liver (BAL) during hemoperfusion. This study was designed to elucidate how immunity may cause death of xenogeneic hepatocytes in the BAL. Healthy dogs were treated with a BAL containing hollow fiber membr...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/000000004783983378
更新日期:2004-01-01 00:00:00
abstract::Testis-derived Sertoli cells have been used to create an immune "privileged" site outside of the testis to facilitate cell transplantation protocols for diabetes and neurodegenerative diseases. In addition to secreting immunoprotective factors, Sertoli cells also secrete growth and trophic factors that appear to enhan...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.1016/s0963-6897(97)00022-5
更新日期:1997-03-01 00:00:00
abstract::Amyotrophic lateral sclerosis (ALS) is a fatal disease of motor neuron degeneration in the brain and spinal cord. Progressive paralysis of the diaphragm and other respiratory muscles leading to respiratory dysfunction and failure is the most common cause of death in ALS patients. Respiratory impairment has also been s...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.1177/0963689720913494
更新日期:2020-01-01 00:00:00
abstract::Gastric cancer (GC) is a leading cause of cancer-related death with poor prognosis. Growing evidence has shown that long noncoding ribonucleic acid (lncRNA) FEZ family zinc finger 1 antisense RNA 1(FEZF1-AS1), an "oncogene," regulates tumor progression and supports cancer stem cell. However, the tumorigenic mechanism ...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.1177/0963689720925059
更新日期:2020-01-01 00:00:00
abstract::Maintenance of freshly isolated porcine liver cells in vitro is limited for a short period of time. Therefore, establishment of easy handling cell lines is extremely important for in vitro study for liver cells and their possible utilization for cell differentiation and growth of stem cells. Porcine liver cells were t...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/096368910X508979
更新日期:2010-01-01 00:00:00
abstract::Stem cells from dental tissues have been isolated and established for tooth regenerative applications. However, basic characterization on their biological properties still needs to be investigated before employing them for effective clinical trials. In this study, we compared the telomere length, relative telomerase ...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/096368911X565001
更新日期:2011-01-01 00:00:00
abstract::Digital ulcers (DUs) are a rather frequent and invalidating complication in systemic sclerosis (SSc), often showing a very slow or null tendency to heal, in spite of the commonly used systemic and local therapeutic procedures. Recently, stem cell therapy has emerged as a new approach to accelerate wound healing. In th...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/096368914X685636
更新日期:2015-01-01 00:00:00
abstract::Endothelial progenitor cells (EPCs) have been applied for cell therapy because of their roles in angiogenesis and neovascularization in ischemic tissue. However, adverse responses caused by EPC therapy have not been fully investigated. In this study, a human peripheral blood sample was collected from a healthy donor a...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.1177/0963689718821686
更新日期:2019-07-01 00:00:00
abstract::For isolated pancreatic islet cell preparations, it is important to be able to reliably assess their mass and quality, and for clinical applications, it is part of the regulatory requirement. Accurate assessment, however, is difficult because islets are spheroid-like cell aggregates of different sizes (<50 to 500 μm) ...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/096368916X691655
更新日期:2016-10-01 00:00:00
abstract::For clinical islet transplantation, isolated islets deteriorate rapidly in culture, although culturing islets prior to transplantation provides flexibility for evaluation of isolated islets and pretreatment of patients. In the present study, we compared human fresh islets to cultured islets with in vitro and in vivo ...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/096368911X605439
更新日期:2012-01-01 00:00:00
abstract::To overcome common disadvantages of standard cultured epidermal sheet grafts (CEG) we have developed a new technique of transplanting cultured human keratinocytes suspended as single cells in a fibrin-glue matrix (Keratinocyte-fibrin-glue suspension-KFGS). In an athymic mouse model with reproducible standardized full ...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.1016/s0963-6897(98)00005-0
更新日期:1998-05-01 00:00:00
abstract::In diabetic patients cardiovascular morbidity and mortality is still a major problem. Our aim was to study the effect of kidney-pancreas transplantation on survival, cardiovascular events, and causes of death in diabetic type 1 uremic patients. Three hundred and thirty-three uremic IDDM patients were enrolled in our w...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.1177/096368970000900621
更新日期:2000-11-01 00:00:00
abstract::We attempted to regenerate bone in a significant osseous defect with various stem cells from deciduous teeth, extracted from puppies, and grafted them into a parent canine mandible as an allograft, parent dental pulp, and bone marrow by tissue engineering and regenerative medicine technology using platelet-rich plasm...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/096368910X539128
更新日期:2011-01-01 00:00:00
abstract::Hepatocyte transplantation is a promising therapy for acute liver failure in humans. Recently, we succeeded in inducing various acute and chronic liver failures in nude mice. Engraftment of transplanted xenogeneic rat hepatocytes, visualized in the host liver by anti-MHC class I immunohistochemistry, revealed that liv...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/096368908785096051
更新日期:2008-01-01 00:00:00
abstract::The present study was designed to evaluate the use of collagen gel loaded with human retinal pigment epithelium (ARPE19) in cellular transfer and to assess its viability within the gel. Collagen solution was prepared by dissolving calfskin in hydrochloric acid to make a final concentration of 2.0 mg/ml and this was mi...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/000000004783983666
更新日期:2004-01-01 00:00:00
abstract::Bone marrow-derived cells have different plastic properties, especially regarding cell fusion, which increases with time and is prompted by tissue injury. Several recessive mutations, including Purkinje Cell Degeneration, affect the number of Purkinje cells in homozygosis; heterozygous young animals have an apparently...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/096368912X638900
更新日期:2012-01-01 00:00:00
abstract::Successful repair of damage in cerebral palsy (CP) needs effective clinical interventions other than simply symptomatic treatments. To elucidate the feasibility of using olfactory ensheathing cells (OECs) to treat CP in children and adolescents, we conducted a randomized controlled clinical trial (RCT) on 33 patients....
journal_title:Cell transplantation
pub_type: 杂志文章,随机对照试验
doi:10.3727/096368910X492652
更新日期:2010-01-01 00:00:00
abstract::Studies of human neurodevelopmental disorders and stem cell-based regenerative transplants have been hampered by the lack of a model of the developing human brain. Stem cell-derived neurons suffer major limitations, including the ability to recapitulate the 3-dimensional architecture of a brain tissue and the represen...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.1177/0963689717752946
更新日期:2018-03-01 00:00:00
abstract::To engineer liver tissues with a clinically significant size, in vivo evaluation of performance using large-scale animal studies are necessary before proceeding to human clinical trials. As pigs are the most suitable candidates, the development of culture conditions suitable for porcine hepatocyte progenitors is very ...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.3727/000000006783981486
更新日期:2006-01-01 00:00:00
abstract::The clinical consequences of acute liver failure are associated with high mortality. Intensive medical intervention is required to treat the symptoms of liver failure, including coagulopathy, metabolic instability, and encephalopathy. Providing temporary liver support with an extracorporeal liver assist device could s...
journal_title:Cell transplantation
pub_type: 杂志文章
doi:10.1177/096368970000900101
更新日期:2000-01-01 00:00:00