Structural Features of the ATP-Binding Cassette (ABC) Transporter ABCA3

被引：27

作者：

Paolini, Alessandro ^{[1
]}

Baldassarre, Antonella ^{[1
]}

Del Gaudio, Ilaria ^{[1
]}

Masotti, Andrea ^{[1
]}

机构：

[1] Bambino Gesu Childrens Hosp IRCCS, Gene Express Microarrays Lab, I-00146 Rome, Italy

来源：

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES | 2015年 / 16卷 / 08期

关键词：

ABC transporters; ABCA3; protein model; SECONDARY STRUCTURE PREDICTION; FATAL SURFACTANT DEFICIENCY; ESCHERICHIA-COLI; LIPID TRANSPORT; II CELLS; PULMONARY SURFACTANT; RESPIRATORY-FAILURE; MEMBRANE-PROTEIN; LAMELLAR BODIES; P-GLYCOPROTEIN;

D O I：

10.3390/ijms160819631

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

In this review we reported and discussed the structural features of the ATP-Binding Cassette (ABC) transporter ABCA3 and how the use of bioinformatics tools could help researchers to obtain a reliable structural model of this important transporter. In fact, a model of ABCA3 is still lacking and no crystallographic structures (of the transporter or of its orthologues) are available. With the advent of next generation sequencing, many disease-causing mutations have been discovered and many more will be found in the future. In the last few years, ABCA3 mutations have been reported to have important pediatric implications. Thus, clinicians need a reliable structure to locate relevant mutations of this transporter and make genotype/phenotype correlations of patients affected by ABCA3-related diseases. In conclusion, we strongly believe that the model preliminarily generated by these novel bioinformatics tools could be the starting point to obtain more refined models of the ABCA3 transporter.

引用

页码：19631 / 19644

页数：14

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