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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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BNIP3L
BCL2 interacting protein 3 like
Chromosome 8 · 8p21.2
NCBI Gene: 665Ensembl: ENSG00000104765.17HGNC: HGNC:1085UniProt: O60238
104PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
negative regulation of programmed cell deathnuclear envelopecellular response to hypoxiaprotein bindingschizophreniahypertensionneurodegenerative diseasemathematical ability
✦AI Summary

BNIP3L (BCL2 interacting protein 3 like) is a mitochondrial outer membrane protein that serves as a critical regulator of mitochondrial homeostasis through multiple cellular pathways. Primary Function: BNIP3L functions as a mitochondrial cargo receptor that mediates mitophagy—the selective autophagic removal of dysfunctional or excess mitochondria 1. It recognizes autophagosomes and facilitates mitochondrial elimination, a process initially characterized during reticulocyte development 1. Additionally, BNIP3L participates in mitochondrial quality control through interaction with SPATA18/MIEAP, mediating mitochondrial-associated degradation of damaged proteins (MALM) 2. Mechanism: BNIP3L induces apoptosis and can overcome anti-apoptotic suppressors BCL-2 and BCL-XL, although high BCL-XL expression inhibits this function [UniProt]. It functions alongside BNIP3 and other mitochondrial cargo receptors in stress-responsive mitophagy, regulated by signaling pathways including AMPK, ATF4, FoxOs, and sirtuins 2. Disease Relevance: BNIP3L-mediated mitophagy is implicated in cancer progression, neurological disorders 1, autoimmune diseases including inflammatory bowel disease and systemic lupus erythematosus 3, age-related macular degeneration 4, and intervertebral disc degeneration 5. Clinical Significance: BNIP3L represents a potential therapeutic target for modulating disease pathways involving mitochondrial dysfunction, particularly in cancer treatment optimization and neurodegenerative disease management.

Sources cited
1
BNIP3L/NIX serves as a mitophagy receptor recognizing autophagosomes; initially identified for clearing mitochondria during reticulocyte development; involved in cancer and neurological disorders
PMID: 34930907
2
BNIP3L is a mitochondrial cargo receptor induced by stress (hypoxia, nutrient deprivation) that targets mitochondria to autophagosomes; regulates mitophagy in tumorigenesis and metastasis
PMID: 33580835
3
BNIP3L/NIX participates in mitophagy pathways that restrict inflammatory cytokine secretion and regulate immune cell homeostasis; implicated in autoimmune diseases including IBD and SLE
PMID: 30951392
4
BNIP3L/NIX involved in selective autophagy and mitochondrial quality control relevant to age-related macular degeneration pathogenesis
PMID: 35468037
5
BNIP3L participates in HIF1A-mediated autophagy axis protecting nucleus pulposus stem cells from compression-induced apoptosis in intervertebral disc degeneration
PMID: 33455530
6
BNIP3L (NIX) mediates mitophagy in mammals during red blood cell differentiation; mitophagy regulated in mammalian cells alongside PINK1 and parkin pathways
PMID: 21179058
Disease Associationsⓘ20
schizophreniaOpen Targets
0.47Moderate
hypertensionOpen Targets
0.44Moderate
neurodegenerative diseaseOpen Targets
0.44Moderate
mathematical abilityOpen Targets
0.29Weak
intelligenceOpen Targets
0.27Weak
metabolic syndromeOpen Targets
0.12Weak
diabetes mellitusOpen Targets
0.12Weak
Abnormality of the immune systemOpen Targets
0.11Weak
type 2 diabetes mellitusOpen Targets
0.10Weak
Miyoshi myopathyOpen Targets
0.09Suggestive
hepatocellular carcinomaOpen Targets
0.09Suggestive
subarachnoid hemorrhageOpen Targets
0.09Suggestive
Blackfan-Diamond anemiaOpen Targets
0.09Suggestive
Beta-thalassemia - X-linked thrombocytopeniaOpen Targets
0.08Suggestive
beta-thalassemia-X-linked thrombocytopenia syndromeOpen Targets
0.08Suggestive
hemoglobin D diseaseOpen Targets
0.07Suggestive
dehydrated hereditary stomatocytosisOpen Targets
0.07Suggestive
Congenital dyserythropoietic anemia type IOpen Targets
0.07Suggestive
dominant beta-thalassemiaOpen Targets
0.07Suggestive
autosomal dominant sideroblastic anemiaOpen Targets
0.07Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ATG12Protein interaction100%SQSTM1Protein interaction100%NBR1Protein interaction100%ATG5Protein interaction100%TP53Protein interaction100%GABARAPL2Protein interaction100%
Tissue Expression6 tissues
Bone Marrow
100%
Brain
90%
Ovary
61%
Heart
38%
Lung
37%
Liver
14%
Gene Interaction Network
Click a node to explore
BNIP3LATG12SQSTM1NBR1ATG5TP53GABARAPL2
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt O60238
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.66LoF Tolerant
pLIⓘ
0.47Tolerant
Observed/Expected LoF0.39 [0.24–0.66]
RankingsWhere BNIP3L stands among ~20K protein-coding genes
  • #4,573of 20,598
    Most Researched104 · top quartile
  • #4,823of 17,882
    Most Constrained (LOEUF)0.66
Genes detectedBNIP3L
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
BNIP3L/NIX-mediated mitophagy: molecular mechanisms and implications for human disease.
PMID: 34930907
Cell Death Dis · 2021
1.00
2
ROS-mediated lysosomal membrane permeabilization and autophagy inhibition regulate bleomycin-induced cellular senescence.
PMID: 38762757
Autophagy · 2024
0.90
3
Autophagy in age-related macular degeneration.
PMID: 35468037
Autophagy · 2023
0.80
4
Hypoxia-induced GPCPD1 depalmitoylation triggers mitophagy via regulating PRKN-mediated ubiquitination of VDAC1.
PMID: 36803235
Autophagy · 2023
0.70
5
Emerging views of mitophagy in immunity and autoimmune diseases.
PMID: 30951392
Autophagy · 2020
0.60