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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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PIPOX
pipecolic acid and sarcosine oxidase
Chromosome 17 · 17q11.2
NCBI Gene: 51268Ensembl: ENSG00000179761.13HGNC: HGNC:17804UniProt: Q9P0Z9
19PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingsarcosine oxidase activityL-pipecolate oxidase activityL-lysine catabolic processmajor depressive disorderagingerectile dysfunctionbipolar disorder
✦AI Summary

PIPOX (pipecolic acid and sarcosine oxidase) is a flavoenzyme that catalyzes the oxidation of L-pipecolate to Δ1-piperideine-6-carboxylate (P6C) as part of the lysine catabolic pathway, with subcellular localization in mitochondria 1. The enzyme plays a critical role in cellular stress protection, as PIPOX knockdown abolishes pipecolate-mediated protection against hydrogen peroxide-induced cell death 1. The protective mechanism involves activation of mTORC1/mTORC2 and Akt signaling pathways, leading to phosphorylation of FoxO3 transcription factor 1. In cancer contexts, PIPOX expression varies significantly across breast cancer subtypes, with highest expression in HER-2 type tumors and lowest in triple-negative breast cancer 2. PIPOX expression is associated with clinical outcomes, as positivity correlates with shorter overall survival in metastatic breast cancer, particularly in lung metastases 3. The enzyme shows higher expression in invasive lobular carcinoma compared to invasive ductal carcinoma and correlates with androgen receptor positivity 4. Additionally, PIPOX has been identified as a fusion partner (TAOK1::PIPOX) in high-grade serous ovarian carcinoma, suggesting potential roles in tumorigenesis 5.

Sources cited
1
PIPOX oxidizes L-pipecolate to P6C, localizes to mitochondria, and is essential for pipecolate-mediated protection against oxidative stress through mTORC1/mTORC2 and Akt signaling
PMID: 27922192
2
PIPOX expression varies across breast cancer subtypes with highest expression in HER-2 type and lowest in triple-negative breast cancer
PMID: 24884785
3
PIPOX positivity correlates with shorter overall survival in metastatic breast cancer, especially lung metastases
PMID: 25550822
4
PIPOX shows higher expression in invasive lobular carcinoma compared to invasive ductal carcinoma and correlates with androgen receptor positivity
PMID: 25837163
5
PIPOX identified as fusion partner (TAOK1::PIPOX) in high-grade serous ovarian carcinoma
PMID: 41072140
Disease Associationsⓘ20
major depressive disorderOpen Targets
0.41Moderate
agingOpen Targets
0.37Weak
erectile dysfunctionOpen Targets
0.32Weak
bipolar disorderOpen Targets
0.24Weak
Low-frequency hearing lossOpen Targets
0.12Weak
Low-frequency sensorineural hearing impairmentOpen Targets
0.12Weak
Romano-Ward syndromeOpen Targets
0.08Suggestive
familial atrial fibrillationOpen Targets
0.07Suggestive
Brugada syndromeOpen Targets
0.06Suggestive
Familial short QT syndromeOpen Targets
0.06Suggestive
Arrhythmogenic right ventricular dysplasiaOpen Targets
0.06Suggestive
atrial fibrillationOpen Targets
0.06Suggestive
hypertrophic cardiomyopathyOpen Targets
0.06Suggestive
Familial progressive cardiac conduction defectOpen Targets
0.06Suggestive
Rare familial disorder with hypertrophic cardiomyopathyOpen Targets
0.05Suggestive
clear cell renal carcinomaOpen Targets
0.05Suggestive
lagophthalmosOpen Targets
0.05Suggestive
Brugada syndrome 9Open Targets
0.05Suggestive
familial sick sinus syndromeOpen Targets
0.05Suggestive
Wolff-Parkinson-White SyndromeOpen Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
AASSShared pathway100%HYKKShared pathway100%SLC25A21Shared pathway100%DMGDHProtein interaction97%AGXTProtein interaction95%ALAS1Protein interaction95%
Tissue Expression6 tissues
Liver
100%
Brain
2%
Ovary
0%
Lung
0%
Bone Marrow
0%
Heart
0%
Gene Interaction Network
Click a node to explore
PIPOXAASSHYKKSLC25A21DMGDHAGXTALAS1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q9P0Z9
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.11LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.85 [0.66–1.11]
RankingsWhere PIPOX stands among ~20K protein-coding genes
  • #14,493of 20,598
    Most Researched19
  • #11,346of 17,882
    Most Constrained (LOEUF)1.11
Genes detectedPIPOX
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Cytotoxicity and Bioimmunological Activity of Poly(2-Isopropenyl-2-oxazoline) Conjugates with Ibuprofen Using 3D Reconstructed Tissue Models.
PMID: 38805352
Biomacromolecules · 2024
1.00
2
Degradable Cell-Adhesive Hybrid Hydrogels by Cross-Linking of Gelatin with Poly(2-isopropenyl-2-oxazoline).
PMID: 39059021
Biomacromolecules · 2024
0.90
3
Expression of sarcosine metabolism-related proteins according to metastatic site in breast cancer.
PMID: 25550822
Int J Clin Exp Pathol · 2014
0.80
4
Implications of differences in expression of sarcosine metabolism-related proteins according to the molecular subtype of breast cancer.
PMID: 24884785
J Transl Med · 2014
0.70
5
Expression of sarcosine metabolism-related proteins in invasive lobular carcinoma: comparison to invasive ductal carcinoma.
PMID: 25837163
Yonsei Med J · 2015
0.60