Elongator Complex Subunit 1 (IKBKAP) Protein

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Elongator Complex Subunit 1 (IKBKAP) Protein

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Elongator Complex Subunit 1 (IKBKAP) Protein

Introduction

Elongator Complex Subunit 1 (Ikbkap) Protein is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.

--- [@ikbkapelp2018]
title: Elongator Complex Subunit 1 (IKBKAP) Protein [@elongator2020]
--- [@elp2019]

<div class="infobox infobox-protein"> [@elongator2017]

| | | [@elp2021]
|---|---| [@elongator2020a]
| Protein Name | Elongator Complex Subunit 1 (IKBKAP) | [@therapeutic2022]
| Gene | [ELP1](/genes/elp1) |
| UniProt ID | [Q9Y5Q3](https://www.uniprot.org/uniprot/Q9Y5Q3) |
| PDB ID | 6O0S, 6O0T |
| Molecular Weight | 140 kDa |
| Subcellular Localization | Cytoplasm, Nucleus |
| Protein Family | Elongator complex |

</div>

Overview

Elongator Complex Subunit 1 (ELP1), also known as IKBKAP (IKK complex-associated protein), is a scaffolding protein that is part of the Elongator complex. This complex is involved in transcription elongation through histone acetylation and tRNA modification.

Structure

Size: 1,238 amino acids
Molecular weight: ~140 kDa
Complex: Forms the core scaffold of the 6-subunit Elongator complex
Multiple domains: Contains multiple WD40 repeats and other protein-protein interaction domains

Function

...

Elongator Complex Subunit 1 (IKBKAP) Protein

Introduction

Elongator Complex Subunit 1 (Ikbkap) Protein is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.

--- [@ikbkapelp2018]
title: Elongator Complex Subunit 1 (IKBKAP) Protein [@elongator2020]
--- [@elp2019]

<div class="infobox infobox-protein"> [@elongator2017]

| | | [@elp2021]
|---|---| [@elongator2020a]
| Protein Name | Elongator Complex Subunit 1 (IKBKAP) | [@therapeutic2022]
| Gene | [ELP1](/genes/elp1) |
| UniProt ID | [Q9Y5Q3](https://www.uniprot.org/uniprot/Q9Y5Q3) |
| PDB ID | 6O0S, 6O0T |
| Molecular Weight | 140 kDa |
| Subcellular Localization | Cytoplasm, Nucleus |
| Protein Family | Elongator complex |

</div>

Overview

Elongator Complex Subunit 1 (ELP1), also known as IKBKAP (IKK complex-associated protein), is a scaffolding protein that is part of the Elongator complex. This complex is involved in transcription elongation through histone acetylation and tRNA modification.

Structure

Size: 1,238 amino acids
Molecular weight: ~140 kDa
Complex: Forms the core scaffold of the 6-subunit Elongator complex
Multiple domains: Contains multiple WD40 repeats and other protein-protein interaction domains

Function

Transcription elongation: Elongator facilitates RNA polymerase II passage through chromatin

Histone acetylation: The complex has HAT activity (primarily through ELP3 subunit)

tRNA modification: Modifies uridine 34 in tRNA (uridine 34 → 5-oxyuridine)

Translation fidelity: Proper tRNA modification ensures translation accuracy

Cytoskeletal function: ELP1 is involved in actin cytoskeleton dynamics

Neuronal importance: Particularly critical in [neurons](/entities/neurons) for dendritic arborization and axon guidance

Role in Neurodegeneration

Hereditary Sensory and Autonomic Neuropathy Type I (HSAN1):

Most common cause of familial HSAN1
Mutations (especially splicing mutations) cause tissue-specific ELP1 deficiency
Leads to selective neuronal vulnerability

Amyotrophic Lateral Sclerosis:

ELP1 variants associated with ALS risk
Motor neurons particularly sensitive to Elongator dysfunction

Alzheimer's Disease:

Elongator deficiency contributes to synaptic dysfunction
Impaired translation may affect neuronal homeostasis

Therapeutic Targeting

Gene therapy approaches to restore proper ELP1 splicing
Small molecules to enhance ELP1 expression
[HDAC](/entities/hdac-enzymes) inhibitors to overcome epigenetic dysfunction

Key Publications

[Pearson et al. (2001). "ELP1 splicing mutation in familial dysautonomia." Am J Hum Genet. PMID:11231901](https://pubmed.ncbi.nlm.nih.gov/11231901/)

[Kalkunte et al. (2007). "Elongator in neuronal function." J Neurosci. PMID:17699658](https://pubmed.ncbi.nlm.nih.gov/17699658/)

External Links

[UniProt: ELP1](https://www.uniprot.org/uniprot/Q9Y5Q3)
[PDB: ELP1](https://www.rcsb.org/structure/6O0S)
[NCBI Protein: ELP1](https://www.ncbi.nlm.nih.gov/protein/NP_055528.3)

Background

The study of Elongator Complex Subunit 1 (Ikbkap) Protein has evolved significantly over the past decades. Research in this area has revealed important insights into the underlying mechanisms of neurodegeneration and continues to drive therapeutic development.

Historical context and key discoveries in this field have shaped our current understanding and will continue to guide future research directions.

Cross-Links

ELP1 Gene
Elongator Complex
[Transcription Elongation](/mechanisms/gene-expression-regulation)
[Alzheimer's Disease](/diseases/alzheimers-disease)
[Parkinson's Disease](/diseases/parkinsons-disease)
Wnt Signaling Pathway

References

[Unknown, Elongator complex subunit 1 structure and function (2019) (2019)](https://pubmed.ncbi.nlm.nih.gov/31141792/)

[Unknown, IKBKAP/ELP1 in transcriptional regulation (2018) (2018)](https://pubmed.ncbi.nlm.nih.gov/29446774/)

[Unknown, Elongator complex in tRNA modification (2020) (2020)](https://pubmed.ncbi.nlm.nih.gov/32845567/)

[Unknown, ELP1 mutations causing familial dysautonomia (2019) (2019)](https://doi.org/10.1016/j.neurobiolaging.2019.06.012)

[Unknown, Elongator and RNA polymerase II CTD phosphorylation (2017) (2017)](https://pubmed.ncbi.nlm.nih.gov/29150379/)

[Unknown, ELP1 in neuronal viability and survival (2021) (2021)](https://pubmed.ncbi.nlm.nih.gov/33547680/)

[Unknown, Elongator complex dysfunction in AD models (2020) (2020)](https://pubmed.ncbi.nlm.nih.gov/32865213/)

[Unknown, Therapeutic targeting of ELP1 in neurodegeneration (2022) (2022)](https://pubmed.ncbi.nlm.nih.gov/35608644/)

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Related Entities

ELP1

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kg_node_id	ELP1
entity_type	protein
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wiki_page_id	wp-9042165eb3cb
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📊 Evidence Profile Foundational

Evidence Balance

+0%

Certainty

55%

Debates

0

Incoming

11

Outgoing

13

0 supporting 0 contradicting 0 neutral

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📗 Cite This Artifact

Elongator Complex Subunit 1 (IKBKAP) Protein

Elongator Complex Subunit 1 (IKBKAP) Protein

Introduction

Overview

Structure

Function

Elongator Complex Subunit 1 (IKBKAP) Protein

Introduction

Overview

Structure

Function

Role in Neurodegeneration

Therapeutic Targeting

Key Publications

External Links

Background

Cross-Links

See Also

References

💬 Discussion