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Kelch-like protein 11 (KLHL11)
Structure:
- contains 1 BACK domain (BTB/Kelch associated domain)
- contains 1 BTB domain (POZ domain)
- contains 5 Kelch repeats
Pathology:
- KLHL11 autoimmunity associated with
- paraneoplastic rhombencephalitis [2,3]*
- testicular germ cell tumors [2,4]
* case associated with Ondine's curse & trismus (images & video)
General
kelch-like protein
Properties
SIZE: MW = 80 kD
entity length = 708 aa
COMPARTMENT: cytoplasm
MOTIF: signal sequence {1-15}
BTB domain {94-170}
MOTIF: Zinc finger
NAME: Zinc finger
EFFECTOR-BOUND: Zn+2
BACK {205-307}
kelch repeat {360-407}
kelch repeat {408-453}
kelch repeat {455-501}
kelch repeat {503-556}
kelch repeat {610-661}
Database Correlations
UniProt Q9NVR0
PFAM correlations
References
- UniProt :accession Q9NVR0
- Devine MF, Gay PC, Dubey D
Ondine's Curse and Trismus in Association With Kelch-like Protein-11
Autoimmunity.
JAMA Neurol. Published online May 28, 2021
PMID: 34047764
https://jamanetwork.com/journals/jamaneurology/fullarticle/2780425
- Dubey D, Wilson MR, Clarkson B et al
Expanded Clinical Phenotype, Oncological Associations, and Immunopathologic
Insights of Paraneoplastic Kelch-like Protein-11 Encephalitis.
JAMA Neurol. 2020 Nov 1;77(11):1420-1429
PMID: 32744608
- Mandel-Brehm C, Dubey D, Kryzer TJ et al
Kelch-like Protein 11 Antibodies in Seminoma-Associated Paraneoplastic
Encephalitis.
N Engl J Med. 2019 Jul 4;381(1):47-54
PMID: 31269365 Free PMC article