Target intelligence / Profile preview

Centriolar satellite-associated tubulin polyglutamylase complex regulator 1 (CSTPP1)

Target
CSTPP1
Molecular classification
Other (Regulator/adaptor protein, cytoskeletal modulatory protein)
01

Overview

Centriolar satellite-associated tubulin polyglutamylase complex regulator 1 (CSTPP1) is a protein primarily involved in regulating the stability and assembly of the tubulin polyglutamylase complex (TPGC), which modulates post-translational modification of microtubules by polyglutamylation[1][2][8]. CSTPP1 supports cytoskeletal organization, controls the shape of the nucleus, and facilitates cilium disassembly by balancing microtubule and actin filament assembly. Loss of CSTPP1 disrupts these processes, resulting in abnormal nuclear morphology, impaired actin organization, and defective ciliogenesis[2]. CSTPP1 is evolutionarily conserved and widely expressed in human tissues, notably in brain and immune cells[5]. It functions mainly as an adaptor protein and does not belong to a common therapeutic target class such as receptor, enzyme, transporter, or ion channel. Clinically, mutations or loss-of-function effects have been associated with mitochondrial complex III deficiency and developmental defects; however, it is not recognized as a pharmacological target, nor are there documented drugs or biomarker utilities directly linked to this protein[1][2][5][8].

Other names
C11orf49FLJ22210MGC4707UPF0705 protein C11orf49centriolar satellite-associated tubulin polyglutamylation protein 1centriolar satellite-associated tubulin polyglutamylase complex regulator 1
02

Biological functions

Cytoskeletal organizationRegulation of protein complex stabilityNuclear shape regulationCilium disassemblyRegulation of microtubule polyglutamylation
03

Disease associations

Mitochondrial complex III deficiencyDevelopmental defectsPotential roles in laminopathies and cancer-associated nuclear morphology

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