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Pedican
Pediatric cancer database
General information | Literature | Expression | Regulation | Mutation | Interaction

Basic Information

Gene ID

50485

Name

SMARCAL1

Synonymous

HARP|HHARP;SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily a-like 1;SMARCAL1;SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily a-like 1

Definition

ATP-driven annealing helicase|HepA-related protein|SMARCA-like protein 1|SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily A-like protein 1|sucrose nonfermenting protein 2-like 1

Position

2q35

Gene type

protein-coding

Cancer type

Abstract

undifferentiated Carcinoma;unclassified

Schimke Immunoosseous Dysplasia (SIOD) is a rare, autosomal recessive disorder of childhood with classical features of spondyloepiphyseal dysplasia, renal failure, and T cell immunodeficiency. SIOD has been associated with several malignancies,including non-Hodgkin lymphoma and osteosarcoma. About half of SIOD patients have biallelic mutations in SMARCAL1 (SWI/SNF-related matrix-associated actin-dependent regulator of chromatin, subfamily a-like 1). This gene encodes an annealing helicase and replication stress response protein that localizes to damage-stalled DNA replication forks. We report a child with SIOD and a novel S859P missense mutation in SMARCAL1 who developed undifferentiated carcinoma of the sinus. Pediatr Blood cancer 2013;60:E88-E90. (c) 2013 Wiley Periodicals, Inc.#CI- Copyright (c) 2013 Wiley Periodicals, Inc.

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