The Alpha One International Registry (AIR), a multinational research program focused on alpha1-antitrypsin (AAT) deficiency, was formed in response to a World Health Organization recommendation. Each of the nearly 20 participating countries maintains a national registry of patients with AAT deficiency and contributes to an international database located in Malmö, Sweden. This database is design...

The most common consequences of acute acoustic trauma (AAT) are hearing loss at frequencies above 3 kHz and tinnitus. In this study, we have used functional Magnetic Resonance Imaging (fMRI) to visualize neuronal activation patterns in military adults with AAT and various tinnitus sequelae during an auditory "oddball" attention task. AAT subjects displayed overactivities principally during refl...

BACKGROUND The development of COPD in subjects with alpha-1 antitrypsin (AAT) deficiency is likely to be influenced by modifier genes. Genome-wide association studies and integrative genomics approaches in COPD have demonstrated significant associations with SNPs in the chromosome 15q region that includes CHRNA3 (cholinergic nicotine receptor alpha3) and IREB2 (iron regulatory binding protein 2...

The degradation and stability of biodegradable films determine the service length mulch in actual use. Most polymers degrade too fast to meet required durability films. objective this work is investigate behaviors poly(butylene adipate-co-terephthalate) (PBAT)/polyhydroxyalkanoate (PHA) blend Several different types stabilizers were incorporated blends provide protection for PHA/PBAT during the...

Over the past two decades, our understanding of the endocannabinoid system has greatly improved due to the wealth of results obtained from exploratory studies. Currently, two cannabinoid receptor subtypes have been well-characterized. The cannabinoid receptor type 1 (CB1) is widely expressed in the central nervous system, while the levels of the cannabinoid receptor type 2 (CB2) in the brain an...

Introduction Alpha-1-antitrypsin deficiency (AATD) is one of the most common genetic disorders, with an estimated prevalence of 1:2000 to 1:5000 in Europe and Scandinavia, depending on the geographic location and type of epidemiologic study [9]. The alpha-1-antitrypsin gene, or SERPINA1 gene (serine proteinase inhibitor), is located on chromosome 14. Patients with the homozygous AAT deficiency ...

Wound healing disturbance is a common complication following surgery, but the underlying cause sometimes remains elusive. A 50-year-old Caucasian male developed an initially misunderstood severe wound healing disturbance following colon and abdominal wall surgery. An untreated alpha-1-antitrypsin (AAT) deficiency in the patient's medical history, known since 20 years and clinically apparent as ...

Alpha-1-Antitrypsin (AAT) deficiency associated panniculitis is commonly included in the dermatologist's differential diagnosis but the disease is rarely ever diagnosed or discussed. The pathophysiology, clinical presentation, histopathology, diagnosis, and treatment of AAT deficiency panniculitis will be reviewed.

Our study investigated alpha 1 antitrypsin deficiency (AATD) diagnosis in a family originated from central Tunisia and showing a familial history of asthma. Biochemical and genetic diagnosis for AATD was performed according to current diagnostic standards. AAT level quantification in affected individuals showed plasma AAT levels consistent with intermediate AATD (ranged from 0.91 to 1.04 g/L). ...

and plays a key role in carbon and nitrogen metabolism in plants. The enzyme has been shown to be involved in the shuttling of reducing equivalents from the cytoplasm to chloroplasts, mitochondria, glyoxysomes, and peroxisomes via the malate-aspartate shuttle (Wightman and Forest, 1978). Up to five separate isoforms of AAT have been reported in plants, differing both kinetically and in levels a...