CYP11B1
Overview
CYP11B1 (Cytochrome P450 Family 11 Subfamily B Member 1, also known as 11-beta-hydroxylase) is a steroidogenic enzyme in the adrenal cortex that catalyzes the final step in cortisol biosynthesis. Its expression is a marker of adrenocortical differentiation and is used to distinguish adrenocortical components in neuroendocrine tumors.
Alterations observed in the corpus
- CYP11B1 overexpressed in the cortical admixture PCC/PGL subtype alongside CYP21A2 and STAR; these adrenal cortex markers define the cortical admixture molecular subtype in 173-patient TCGA PCC/PGL cohort PMID:28162975
Cancer types (linked)
- PHC / PGNG: CYP11B1 expression is highest in the cortical admixture PCC/PGL subtype, which also carries rare germline MAX mutations (p<0.032); expression-based subtype marker PMID:28162975
Co-occurrence and mutual exclusivity
- CYP11B1 overexpression co-occurs with CYP21A2 and STAR overexpression in the cortical admixture subtype; germline MAX mutations are enriched in this subtype PMID:28162975
Therapeutic relevance
- CYP11B1 is a subtype marker rather than a direct therapeutic target in PCC/PGL in this study.
Open questions
- Whether the cortical admixture subtype’s adrenocortical marker expression reflects multifocal disease, tissue-sampling artifact, or a distinct biological state requires further investigation PMID:28162975
Sources
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