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家族性复发性髌骨脱位的解剖学特征及潜在基因突变位点

Anatomical characteristics and potential gene mutation sites of a familial recurrent patellar dislocation.

机构信息

Department of Orthopedics, Huizhou Central People's Hospital, Huizhou, 516008, Guangdong, People's Republic of China.

出版信息

BMC Med Genomics. 2022 Aug 7;15(1):176. doi: 10.1186/s12920-022-01330-9.

Abstract

BACKGROUND

Recurrent patellar dislocation is the result of anatomical alignment and imbalance of restraint of bone and soft tissue. We investigate the anatomical characteristics of the knee joint in a family of patients with recurrent patella dislocation, and to screen the possible pathogenic genes in this family by whole exome sequencing in 4 patients and 4 healthy subjects, so as to provide theoretical basis for the pathogenesis of this disease.

METHODS

The data related to patella dislocation were measured by imaging data. The peripheral blood DNA of related family members was extracted for the whole exome sequencing, and then the sequencing results were compared with the human database. By filtering out synonymous variants and high-frequency variants in population databases, and then integrating single nucleotide non-synonymous variants of family members, disease-causing genes were found.

RESULTS

All patients in this family have different degrees of abnormal knee anatomy, which is closely related to patella dislocation. The sequencing results of patients and normal persons in this patella dislocation family were compared and analyzed, and the data were filtered through multiple biological databases. Find HOXB9 (NM_024017.4:c.404A>G:p.Glu135Gly),COL1A1(NM_000088.3:c.3766G>A:p.Ala1256Thr),GNPAT(NM_014236.3:c1556A>G:p.Asp519Gly),NANS(NM_018946.3:c.204G>C:p.Glu68Asp),SLC26A2(NM_000112.3:c.2065A>T:p.Thr689Ser) are nonsynonymous variants (MISSENSE). Through Sanger sequencing, the identified mutations in HOXB9 and SLC26A2 genes were only present in samples from patients with recurrent patellar dislocation.

CONCLUSIONS

The patients with recurrent patellar dislocation had markedly abnormal knee anatomy in this family. HOXB9 gene and SLC26A2 gene were found to be the possible pathogenic genes or related genes for patella dislocation.

摘要

背景

复发性髌骨脱位是骨骼和软组织的解剖排列和约束失衡的结果。我们研究了一组复发性髌骨脱位患者的膝关节解剖特征,并对 4 名患者和 4 名健康受试者进行全外显子组测序,以筛选该家族中可能的致病基因,为该病的发病机制提供理论依据。

方法

通过影像学数据测量与髌骨脱位相关的数据。提取相关家族成员的外周血 DNA 进行全外显子组测序,然后将测序结果与人类数据库进行比较。通过过滤同义变体和人群数据库中的高频变体,然后整合家族成员的单核苷酸非同义变体,找到致病基因。

结果

该家系所有患者均有不同程度的膝关节解剖异常,与髌骨脱位密切相关。比较分析髌骨脱位家系患者和正常人的测序结果,通过多个生物学数据库进行数据过滤。在 HOXB9(NM_024017.4:c.404A>G:p.Glu135Gly)、COL1A1(NM_000088.3:c.3766G>A:p.Ala1256Thr)、GNPAT(NM_014236.3:c1556A>G:p.Asp519Gly)、NANS(NM_018946.3:c.204G>C:p.Glu68Asp)、SLC26A2(NM_000112.3:c.2065A>T:p.Thr689Ser)中发现非同义变异(错义)。通过 Sanger 测序,仅在复发性髌骨脱位患者的样本中发现 HOXB9 和 SLC26A2 基因的鉴定突变。

结论

该家系复发性髌骨脱位患者膝关节解剖明显异常。HOXB9 基因和 SLC26A2 基因被认为是髌骨脱位的可能致病基因或相关基因。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fa79/9358890/e4d79ecd0f5a/12920_2022_1330_Fig1_HTML.jpg

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