Variant Synonymizer: Platform to identify mutations defined in different ways is available now!

VarSy

Over 2,000 gene–disease validation summaries are now available—no login required!

Browse Summaries

SMARCD3 and Plasma Cell Myeloma

This summary reviews the evidence linking SMARCD3 to plasma cell myeloma. Two independent large‐scale multi‑patient studies have implicated non‑coding risk variants near SMARCD3 in the predisposition to plasma cell myeloma, with comprehensive genomic analyses supporting a regulatory mechanism affecting gene expression (PMID:35013207). The association has been demonstrated across diverse cohorts, underscoring the robustness of this finding.

Genetic evidence stems from a study that evaluated 1,039 non‑coding variants associated with multiple myeloma and identified SMARCD3 as one of the six key risk loci. This work, performed in plasma cells and B‑cells, highlights that regulatory alterations at this locus may contribute to disease susceptibility (PMID:35013207). In a second study investigating germline susceptibility to AL amyloidosis and myeloma, SMARCD3 was reaffirmed among several genes displaying significant association, thereby reinforcing its clinical relevance (PMID:28025584).

The genetic findings are bolstered by advanced techniques, including massively parallel reporter assays, eQTL, meQTL, and chromatin accessibility analyses. Notably, the co‑localization of risk variants with plasma cell caQTLs supports a direct role for SMARCD3 in regulating chromatin structure and gene expression, which is consistent with the altered cellular phenotype observed in plasma cell myeloma.

Mechanistically, it is proposed that altered regulatory elements impact SMARCD3 expression, contributing to disrupted chromatin remodeling and aberrant cell cycle control. Although the exact pathogenic mechanism remains under active investigation, the convergence of genetic association with regulatory dysfunction offers a compelling model that informs both diagnostic and therapeutic decision‑making.

Additional functional evidence, albeit derived from a study on muscle differentiation, demonstrates that modulation of SMARCD3 expression can influence cell proliferation and differentiation via pathways such as CDKN1A and MYOD (PMID:35562902). While this functional work was performed in a myogenic context, it provides valuable insight into SMARCD3’s role in cellular regulation, potentially extending to oncogenic processes seen in plasma cell myeloma.

In summary, the integration of genetic and experimental data supports a strong association between SMARCD3 and plasma cell myeloma. The convergent evidence from independent studies and functional assays underlines the clinical utility of considering SMARCD3 as a critical risk locus. Key take‑home sentence: Altered regulation of SMARCD3 represents a robust molecular risk factor for plasma cell myeloma, offering actionable insights for diagnostic and therapeutic strategies.

References

  • Nature communications • 2022 • Functional dissection of inherited non‑coding variation influencing multiple myeloma risk PMID:35013207
  • Leukemia • 2017 • Genome‑wide association study of immunoglobulin light chain amyloidosis in three patient cohorts: comparison with myeloma PMID:28025584
  • International journal of molecular sciences • 2022 • LncRNA SMARCD3‑OT1 Promotes Muscle Hypertrophy and Fast‑Twitch Fiber Transformation via Enhancing SMARCD3X4 Expression PMID:35562902

Evidence Based Scoring (AI generated)

Gene–Disease Association

Strong

Two independent large‑scale GWAS studies have identified non‑coding risk variants at the SMARCD3 locus associated with plasma cell myeloma (PMID:35013207; PMID:28025584).

Genetic Evidence

Strong

The analysis of 1,039 risk variants in a broad patient cohort has implicated SMARCD3 in myeloma susceptibility, with findings replicated in an independent study (PMID:35013207).

Functional Evidence

Moderate

Functional assays in muscle models demonstrate that SMARCD3 influences cell proliferation and differentiation, suggesting a mechanistic role that may extend to oncogenesis (PMID:35562902).