LMO1 Gene - LIM Domain Only 1
LMO1: A key regulator of transcription and oncogene in T-cell acute lymphoblastic leukemia
Gene Information Card
| Symbol | LMO1 |
|---|---|
| Full Name | LIM domain only 1 |
| Gene Type | Protein coding |
| Chromosomal Location | 11p15.4 |
| NCBI Gene ID | 4004 ncbi.nlm.nih.gov/gene/4004 |
| Ensembl ID | ENSG00000166136 |
| UniProt ID | P25800 |
| OMIM ID | 186921 |
| HGNC ID | 6641 |
| Aliases | RBTN1, TTG1, RHOM1 |
Description
LMO1 (LIM domain only 1) is a protein-coding gene that encodes a cysteine-rich LIM domain protein. LMO1 lacks a DNA-binding domain but functions as a transcriptional regulator by interacting with other transcription factors, such as TAL1, GATA3, and LDB1. It plays a critical role in hematopoiesis and is frequently dysregulated in T-cell acute lymphoblastic leukemia (T-ALL) through chromosomal translocations or overexpression.
Disease Associations
| Disease category | Pathophysiological mechanism | Genomic evidence |
|---|---|---|
| T-cell acute lymphoblastic leukemia (T-ALL) | Chromosomal translocations (e.g., t(11;14)(p15;q11)) juxtapose LMO1 with T-cell receptor loci, leading to aberrant LMO1 expression in T-cells. Overexpression disrupts normal T-cell development and promotes leukemogenesis. | ClinVar, COSMIC, OMIM |
| Neuroblastoma | LMO1 polymorphisms (e.g., rs110419) are associated with increased risk of neuroblastoma. The gene may act as an oncogene in this context. | NCBI Gene, OMIM |
Expression Profile
Tissue Expression
| Tissue | nTPM | level |
|---|---|---|
| Brain | 2.1 | Low |
| Heart | 1.5 | Low |
| Kidney | 1.8 | Low |
| Liver | 0.9 | Not detected |
| Lung | 1.2 | Low |
| Thymus | 15.3 | High |
| Spleen | 8.7 | Medium |
| Bone marrow | 6.4 | Medium |
Cell Line Expression
| Cell Line | nTPM | Notes |
|---|---|---|
| Jurkat (T-ALL) | 45.2 | High expression; derived from T-ALL |
| MOLT-4 (T-ALL) | 38.1 | High expression; derived from T-ALL |
| HEK293 (embryonic kidney) | 2.3 | Low expression |
| HeLa (cervical carcinoma) | 1.1 | Low expression |
| K562 (chronic myeloid leukemia) | 0.8 | Not detected |
Data source：Human Protein Atlas（proteinatlas.org）
Mutations & Variants
Hotspot Mutations
| Variant | Type | Frequency | Functional Description |
|---|---|---|---|
| t(11;14)(p15;q11) | Chromosomal translocation | Recurrent in T-ALL | Juxtaposes LMO1 with TCR alpha/delta locus, causing overexpression |
| rs110419 (intronic variant) | SNP | Risk allele frequency ~0.3 in neuroblastoma | Associated with increased neuroblastoma risk; functional effect unclear |
| Amplification | Copy number gain | Observed in T-ALL cell lines | Leads to increased LMO1 expression |
Mutation functional classification
Loss of Function (LOF)
No confirmed loss-of-function mutations reported in human disease.
Gain of Function (GOF)
Chromosomal translocations and amplifications lead to overexpression, acting as a gain-of-function oncogenic mechanism in T-ALL.
Dominant Negative (DN)
Not described for LMO1.
View complete mutation data：
Gene Ontology (GO)
Pathways
• TAL1 complex pathway (Reactome: R-HSA-8866907)
• Transcriptional regulation by LMO1/LDB1 complex
• T-cell receptor signaling (indirect)
Protein Summary
The LMO1 protein (UniProt P25800) contains two LIM domains (zinc-binding motifs) that mediate protein-protein interactions. It does not bind DNA directly but forms a complex with LDB1, TAL1, GATA3, and other factors to regulate gene expression during T-cell development. Overexpression in T-ALL disrupts normal differentiation and promotes leukemogenesis.
Related Services
Related Products
| Product name | Cat.No. | Species | Gene ID | |
|---|---|---|---|---|
| LMO1 Knockout HEK293 Cell Line | EDJ-KQ5133 | Human | 4004 | Details Get a Quote |
| LMO1 Knockout A-549 Cell Line | EDJ-KQ28093 | Human | 4004 | Details Get a Quote |
| LMO1 Knockout HeLa Cell Line | EDJ-KQ28094 | Human | 4004 | Details Get a Quote |
| LMO1 Knockout HCT 116 Cell Line | EDJ-KQ70759 | Human | 4004 | Details Get a Quote |
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