<scp> <i>FLNA</i> </scp> Variants Related to Melnick‐Needles Syndrome: Two Mexican Case Reports and a Comprehensive Variant Review

Melnick-Needles Syndrome (MNS; OMIM #309350) is a rare X-linked dominant osteochondrodysplasia caused by FLNA gain-of-function variants. It is characterized by short stature, facial dysmorphism, skeletal anomalies, and systemic complications. FLNA encodes Filamin A, a cytoskeletal protein with over 90 binding partners. Variants in FLNA cause a broad spectrum of disorders, among which MNS represents one of the most severe phenotypes. Despite increasing research on MNS, many aspects of its genetic and phenotypic spectrum remain unknown. In this work, we presented two Mexican cases with a diagnosis of MNS with different FLNA missense variants p.(Leu1193Pro) and p.(Ser1199Leu). In addition, we performed a comprehensive review of the literature about FLNA variants related to MNS. This study reinforced the genetic and phenotypic complexity of MNS, emphasizing the critical role of exon 22 variants in the pathogenesis of the disease.

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Journal
American Journal of Medical Genetics Part A
Published
2025-09-25
DOI
https://doi.org/10.1002/ajmg.a.64246
Primary Topic
Connective tissue disorders research
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article
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article

FLNA Variants Related to Melnick‐Needles Syndrome: Two Mexican Case Reports and a Comprehensive Variant Review

Frank Rauch, Ghalib Bardai, Carlos A. Guzmán-Martín, María Hortensia Valdez‐de la Torre et al.
American Journal of Medical Genetics Part A
Connective tissue disorders research
article

FLNA Variants Related to Melnick‐Needles Syndrome: Two Mexican Case Reports and a Comprehensive Variant Review

Frank Rauch, Ghalib Bardai, Carlos A. Guzmán-Martín, María Hortensia Valdez‐de la Torre, Fernando Ramírez‐Jiménez, Thania Ordaz‐Robles, Ilan Vinitzky Brener, Jessica Vanesa Sánchez‐Aguilar, Sergio Saldaña‐Pimentel
article en

Abstract

Melnick-Needles Syndrome (MNS; OMIM #309350) is a rare X-linked dominant osteochondrodysplasia caused by FLNA gain-of-function variants. It is characterized by short stature, facial dysmorphism, skeletal anomalies, and systemic complications. FLNA encodes Filamin A, a cytoskeletal protein with over 90 binding partners. Variants in FLNA cause a broad spectrum of disorders, among which MNS represents one of the most severe phenotypes. Despite increasing research on MNS, many aspects of its genetic and phenotypic spectrum remain unknown. In this work, we presented two Mexican cases with a diagnosis of MNS with different FLNA missense variants p.(Leu1193Pro) and p.(Ser1199Leu). In addition, we performed a comprehensive review of the literature about FLNA variants related to MNS. This study reinforced the genetic and phenotypic complexity of MNS, emphasizing the critical role of exon 22 variants in the pathogenesis of the disease.

American Journal of Medical Genetics Part AVol. 200(2)
Shriners Hospitals for Children - Canada (CA), Shriners Hospitals for Children - Mexico (MX), Instituto Nacional de Enfermedades Respiratorias (MX), Universidad de Colima (MX)
Openalex Percentile: Top 72%
Connective tissue disorders research
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