TorsinA in PC12 cells: localization in the endoplasmic reticulum and response to stress.

Most cases of early-onset torsion dystonia are caused by deletion of GAG in the coding region of the DYT1 gene encoding torsinA. This autosomal dominant neurologic disorder is characterized by abnormal movements, believed to originate from neuronal dysfunction in the basal ganglia of the human brain. The torsins (torsinA and torsinB) are members of the "ATPases associated with a variety of cellular activities" (AAA(+)) superfamily of proteins that mediate chaperone and other functions involved in conformational modeling of proteins, protection from stress, and targeting of proteins to cellular organelles.

Abdominal aortic aneurysms: cost-effectiveness of elective endovascular and open surgical repair.

Purpose: To evaluate the cost-effectiveness of elective endovascular and open surgical repair of infrarenal abdominal aortic aneurysms (AAAs) by taking into account short- and long-term outcomes.

Materials And Methods: A Markov decision model was developed to evaluate quality-adjusted life-years (QALYs) and lifetime costs of endovascular and open surgical repair. The incremental cost-effectiveness ratio (CER) was calculated for endovascular repair relative to open surgery in a cohort of 70-year-old men with an AAA between 5 and 6 cm in diameter.

Thoracoabdominal aneurysm repair: results with 337 operations performed over a 15-year interval.

Objective: To review perioperative results and late survival after thoracoabdominal aneurysm repair (TAA), in particular to assess the impact over time of epidural cooling (EC) on spinal cord ischemic complications (SCI).

Summary Background Data: A variety of operative approaches and protective adjuncts have been used in TAA to minimize the major complications of perioperative death and SCI. There is no consensus with respect to the optimal approach.

T(H)2 predominant immune responses prevail in human abdominal aortic aneurysm.

T lymphocytes localize within lesions of two diametrically opposed expressions of atherosclerosis: stenosis-producing plaques and ectasia-producing abdominal aortic aneurysm (AAA). T(H)1 immune responses appear to predominate in human stenotic lesions. However, little information exists regarding the nature of the T-cell infiltrate in AAAs.

Clinical failures of endovascular abdominal aortic aneurysm repair: incidence, causes, and management.

Objective: Despite well-documented good early results and benefits of endoluminal stent graft repair of abdominal aortic aneurysm (J Vasc Surg 2002;35:1137-44.)(AAA), the long-term outcome of this method of treatment remains uncertain. In particular, concern exists that late effectiveness and durability are inferior to that of open repair.

Stroop performance in normal control subjects: an fMRI study.

In an attempt to clarify regional signal intensity changes, which may accompany the performance of the Stroop Color-Word task, healthy subjects were imaged using the fMRI BOLD technique while performing a modified version of the task. Both the AAA and VOA subdivisions of the anterior cingulate cortex were significantly activated during the interference condition; however, only the signal intensity change within the VOA correlated with task performance. Additionally, signal intensity change was significantly increased in the VOA subdivision of the cingulate cortex when controlling for signal intensity change present during the performance of a color naming task.

The AAA ATPase Cdc48/p97 and its partners transport proteins from the ER into the cytosol.

In eukaryotic cells, incorrectly folded proteins in the endoplasmic reticulum (ER) are exported into the cytosol and degraded by the proteasome. This pathway is co-opted by some viruses. For example, the US11 protein of the human cytomegalovirus targets the major histocompatibility complex class I heavy chain for cytosolic degradation.

SAP97 interacts with Kv1.5 in heterologous expression systems.

PDZ domain-containing proteins such as SAP97 and ZO-1 have been implicated in the targeting and clustering of ion channels. We have explored the interactions of these polypeptides with a cardiac voltage-gated potassium channel. Immunocytochemistry in cardiac myocytes revealed colocalization of SAP97 and Kv1.

Regression of perianeurysmal fibrosis and ureteral dilation following endovascular repair of inflammatory abdominal aortic aneurysm.

An inflammatory component to abdominal aortic aneurysms (AAA) is thought to occur in approximately 5% of cases. Accompanying ureteral entrapment may be involved in 20% of these. Transabdominal repair of inflammatory AAA with ureterolysis may result in increased complications.

TorsinA: movement at many levels.

TorsinA is the causative protein in the human neurologic disease early onset torsin dystonia, a movement disorder involving dysfunction in the basal ganglia without apparent neurodegeneration. Most cases result from a dominantly acting three-base pair deletion in the TOR1A gene causing loss of a glutamic acid near the carboxyl terminus of torsinA. Torsins are members of the AAA(+) superfamily of ATPases and are present in all multicellular organisms.

Inpatient costs of routine endovascular repair of abdominal aortic aneurysm.

Rationale And Objectives: The purpose of this study was to determine the inpatient cost of routine (ie, without emergent conversion to open repair during the hospital stay) endovascular stent-graft placement in a consecutive series of patients undergoing elective endovascular repair of abdominal aortic aneurysm (AAA) at a single institution.

Materials And Methods: Inpatient hospital costs of 91 patients who underwent initial elective endovascular repair of AAA were analyzed retrospectively. All patients had participated in clinical trials at the authors' institution during the previous 6 years.

Rvb1p and Rvb2p are essential components of a chromatin remodeling complex that regulates transcription of over 5% of yeast genes.

Eukaryotic Rvb1p and Rvb2p are two highly conserved proteins related to the helicase subset of the AAA+ family of ATPases. Conditional mutants in both genes show rapid changes in the transcription of over 5% of yeast genes, with a similar number of genes being repressed and activated. Both Rvb1p and Rvb2p are required for maintaining the induced state of many inducible promoters.

Mutant torsinA, responsible for early-onset torsion dystonia, forms membrane inclusions in cultured neural cells.

Early-onset torsion dystonia is a hereditary movement disorder thought to be caused by decreased release of dopamine into the basal ganglia, without apparent neuronal degeneration. Recent cloning of the gene responsible for this disease, TOR1A (DYT1), identified the encoded protein, torsinA, as a member of the AAA+ superfamily of chaperone proteins and revealed highest levels of expression in dopaminergic neurons in human brain. Most cases of this disease are caused by a deletion of one glutamic acid residue in the C-terminal region of the protein.