BIRC3

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Associated Genetic Biomarkers

Associated Diseases

Overview

Location [1]

11q22.2

Protein [2]

Baculoviral IAP repeat-containing protein 3

Synonyms [1]

API2, RNF49, MALT2, c-IAP2, MIHC, AIP1, HIAP1, HAIP1, CIAP2

Baculovial IAP repeat containing 3 (BIRC3) is a gene that encodes encodes the protein cellular inhibitor of apoptosis protein 2 (cIAP2), which inhibits apoptosis. BIRC3 is localized to chromosome 11q22, whose deletion is associated with high risk disease in patients with chronic lymphocytic leukemia. BIRC3 mutations are observed in <5% of patients with CLL (PMID: 24943832; PMID: 24597984; PMID: 24217197; PMID: 22308293; PMID: 25605254), ~11% of patients with Splenic marginal zone lymphoma (PMID: 21881048) and 6-10% of mantle cell lymphoma cases (PMID: 24362935).

BIRC3 is altered in 0.82% of all cancers with breast invasive ductal carcinoma, cutaneous melanoma, lung adenocarcinoma, colon adenocarcinoma, and head and neck squamous cell carcinoma having the greatest prevalence of alterations [3].

BIRC3 GENIE Cases - Top Diseases

The most common alterations in BIRC3 are BIRC3 Mutation (0.35%), BIRC3 Amplification (0.21%), BIRC3 Loss (0.07%), BIRC3 Frameshift (0.02%), and BIRC3 Nonsense (0.02%) [3].

BIRC3 GENIE Cases - Top Alterations

Biomarker-Directed Therapies

View Therapies for BIRC3

BIRC3 is a predictive biomarker for use of chlorambucil and rituximab in patients.

Marginal zone lymphoma and mature B-cell lymphoma/leukemia have the most therapies with BIRC3 as a predictive biomarker.

Of the therapies with BIRC3 as a predictive biomarker, 0 are FDA-approved in at least one clinical setting and 2 have NCCN guidelines in at least one clinical setting.

BIRC3-MALT1 Fusion and BIRC3 Mutation are the top alterations on BIRC3 targeted by therapies [4].

Chlorambucil + Rituximab +

Marginal Zone Lymphoma -

Biomarker Criteria:

Sample must match all of the following:

BIRC3-MALT1 Fusion

Predicted Response: Primary Sensitivity

Clinical Setting(s): Refractory (NCCN)

Note: Marginal Zone Lymphoma (MZL) is a type of Mature B-Cell Lymphoma. MZL has further subtypes, of which the pulmonary and extranodal MZL of gastric MALT lymphomas frequently carries the t(11;18)(q21;q21) translocation. It is associated with disseminated disease and resistance to antibiotic treatment for H.pylori. The fusion was however associated with improved remission rates in patients treated with a combination of rituximab plus chlorambucil.

Biomarker Criteria: Sample must match all of the following: BIRC3-MALT1 Fusion	Predicted Response: Primary Sensitivity
	Clinical Setting(s): Refractory (NCCN)
	Note: Marginal Zone Lymphoma (MZL) is a type of Mature B-Cell Lymphoma. MZL has further subtypes, of which the pulmonary and extranodal MZL of gastric MALT lymphomas frequently carries the t(11;18)(q21;q21) translocation. It is associated with disseminated disease and resistance to antibiotic treatment for H.pylori. The fusion was however associated with improved remission rates in patients treated with a combination of rituximab plus chlorambucil.

Mature B-Cell Lymphoma/Leukemia -

Biomarker Criteria:

Sample must match all of the following:

BIRC3-MALT1 Fusion

Predicted Response: Primary Sensitivity

Clinical Setting(s): Refractory (NCCN)

Note: A subtype of Mature B-Cell Lymphoma is Marginal Zone Lymphoma (MZL). MZL has further subtypes, of which the pulmonary and extranodal MZL of gastric MALT lymphomas frequently carries t(11;18)(q21;q21) translocation. It is associated with disseminated disease and resistance to antibiotic treatment for H.pylori. The fusion was however associated with improved remission rates in patients treated with a combination of rituximab plus chlorambucil.

Biomarker Criteria: Sample must match all of the following: BIRC3-MALT1 Fusion	Predicted Response: Primary Sensitivity
	Clinical Setting(s): Refractory (NCCN)
	Note: A subtype of Mature B-Cell Lymphoma is Marginal Zone Lymphoma (MZL). MZL has further subtypes, of which the pulmonary and extranodal MZL of gastric MALT lymphomas frequently carries t(11;18)(q21;q21) translocation. It is associated with disseminated disease and resistance to antibiotic treatment for H.pylori. The fusion was however associated with improved remission rates in patients treated with a combination of rituximab plus chlorambucil.

Clinical Trials

View Clinical Trials for BIRC3

Significance of BIRC3 in Diseases

Marginal Zone Lymphoma +

Mature B-Cell Lymphoma/Leukemia +

Lymphoma +

Acute Lymphoblastic Leukemia +

Acute Myeloid Leukemia +

Chronic Lymphocytic Leukemia +

Chronic Myeloid Leukemia +

Multiple Myeloma +

Myelodysplastic Syndromes +

Prolymphocytic Leukemia +

References

1. Hart R and Prlic A. Universal Transcript Archive Repository. Version uta_20180821. San Francisco CA: Github;2015. https://github.com/biocommons/uta

2. The UniProt Consortium. UniProt: a worldwide hub of protein knowledge. Nucleic Acids Research. 2019;47:D506-D515.

3. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

4. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.

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