objective(s): non-syndromic sensorineural hearing loss (nshl) is a common disorder affecting approximately 1 in 500 newborns. this type of hearing loss is extremely heterogeneous and includes over 100 loci. mutations in the gjb2 gene have been implicated in about half of autosomal recessive nshl (arnshl) cases, making this the most common cause of arnshl. for the latter form of deafness, most f...

objective(s)marfan syndrome (mfs) is a severe connective tissue disorder withan autosomal dominant inheritance pattern. early diagnosis is critical in mfs. because of the large size of fibrillin-1 gene (fbn1), the uniqueness of mutations, and the absence of genotype-to-phenotype correlations linkage analysis can be very helpful for early diagnosis of mfs. in this study, eight polymorphic marker...

background: hearing loss (hl) is the most frequent neurosensory impairment. hl is highly heterogeneous defect. this disorder affects 1 out of 500 newborns. this study aimed to determine the role of dfnb2 locus and frequency of myo7a gene mutations in a population from west of iran. methods: thirty families investigated in shahrekord university of medical sciences in 2014, genetic linkage analys...

In order to obtain more accurate predicted dynamic steady-state performance with shorter computation time, an available mathematical model is modified and presented. Using this modified model, performance of a typical switched reluctance motor (SRM) under a wide range of variations of operating conditions is obtained and discussed. These include variations of speed, voltage, load and switching ...

this paper presents a novel salient pole synchronous generator i.e. permanentmagnet-assisted salient-pole synchronous generator (pma-sgs). due to saturationof conventional synchronous generators (sgs), permanent-magnet-assisted salientpolesynchronous generators (pma-sgs) are presented. pma-sgs are a new type ofsalient-pole synchronous machines with extra permanent magnets (pms) betweenthe adjac...

A reduced order numerical computational method based on flux tube modelling is proposed for the rapid electromagnetic analysis and design of electromechanical energy transducers using an example of a synchronous reluctance machine. The flux tube method is applied to establish flux linkage functions facilitating fast and accurate inductance estimation. The practical advantage is that the approac...

abstract background: autosomal dominant polycystic kidney disease (adpkd) is an inherited disorder with genetic heterogeneity. up to three loci are involved in this disease, pkdi on chromosome 16p13.3, pkd2 on 4q21, and a third locus of unknown location. methods: here we report the first molecular genetic study of adpkd and the existence oflocus heterogeneity for adpkd in the iranian population...

background: hearing loss (hl) is the most frequent sensory birth defect in humans. autosomal recessive non-syn­dromic hl (arnshl) is the most common type of hereditary hl. it is extremely heterogeneous and over 70 loci (known as dfnb) have been identified. this study was launched to determine the relative contribution of more frequent loci in a cohort of arnshl families. methods: thirty-seven i...

This paper investigates an improved stator flux linkage observer for sensorless permanent magnet synchronous motor (PMSM) drives using a voltage-based flux linkage model and an adaptive sliding mode variable structure. We propose a new observer design that employs an improved sliding mode reaching law to achieve better estimation accuracy. The design includes two models and two adaptive estimat...

sensorineural non-syndromic hearing loss is the most common disorder which affects 1 in 500 newborns. hearing loss is an extremely heterogeneous defect with more than 100 loci identified to date. according to the studies, mutations in gjb2 are estimated to be involved in 50- 80% of autosomal recessive non-syndromic hearing loss cases, but contribution of other loci in this disorder is yet ambig...