COQ8B Membrane Protein Introduction

Introduction of COQ8B

COQ8B belongs to the protein kinase superfamily which is a structurally conserved protein domain containing the catalytic function of protein kinases. At the same time, it is also a member of the ADCK protein kinase family containing ADCK3 and ADCK4(COQ8B). AraF domain containing kinases 3 and 4 (ADCK3 and ADCK4, respectively) are paralogous human mitochondrial proteins, coorthologs of the yeast protein Coq8 and bacterial protein UbiB. COQ8B is encoded by the COQ8B gene which is located at 19q13.2. The molecular weight of COQ8B is 60,069 Da.

Basic Information of COQ8B
Protein Name	Atypical kinase COQ8B, mitochondrial
Gene Name	COQ8B
Aliases	AarF domain-containing protein kinase 4, Coenzyme Q protein 8B
Organism	Homo sapiens (Human)
UniProt ID	Q96D53
Transmembrane Times	Single-pass membrane
Length (aa)	544
Sequence	MWLKVGGLLRGTGGQLGQTVGWPCGALGPGPHRWGPCGGSWAQKFYQDGPGRGLGEEDIRRAREARPRKTPRPQLSDRSRERKVPASRISRLANFGGLAVGLGLGVLAEMAKKSMPGGRLQSEGGSGLDSSPFLSEANAERIVQTLCTVRGAALKVGQMLSIQDNSFISPQLQHIFERVRQSADFMPRWQMLRVLEEELGRDWQAKVASLEEVPFAAASIGQVHQGLLRDGTEVAVKIQYPGIAQSIQSDVQNLLAVLKMSAALPAGLFAEQSLQALQQELAWECDYRREAACAQNFRQLLANDPFFRVPAVVKELCTTRVLGMELAGGVPLDQCQGLSQDLRNQICFQLLTLCLRELFEFRFMQTDPNWANFLYDASSHQVTLLDFGASREFGTEFTDHYIEVVKAAADGDRDCVLQKSRDLKFLTGFETKAFSDAHVEAVMILGEPFATQGPYDFGSGETARRIQDLIPVLLRHRLCPPPEETYALHRKLAGAFLACAHLRAHIACRDLFQDTYHRYWASRQPDAATAGSLPTKGDSWVDPS

Function of COQ8B Membrane Protein

COQ8B is widely expressed in human, and is also expressed in podocytes and localizes to mitochondria and foot processes and interacts with COQ6 endogenously. In human podocytes, COQ8B interacted with members of the CoQ10 biosynthesis pathway, including COQ6, which has been linked with SRNS and COQ7. COQ8B can interact selectively and non-covalently with ATP, it also owns kinase activity. What’ s more, COQ8B takes part in cerebellar Purkinje cell layer morphogenesis and ubiquinone biosynthetic process. It is required in the biosynthesis of coenzyme Q, a lipid-soluble electron carrier well known for its role in energy metabolism, more specifically in the electron transport chain. Beyond that, mutations in COQ8B cause steroid-resistant nephrotic syndrome, and are also associated with coenzyme Q deficiency. In term of structure, COQ8B adopts an atypical protein kinase-like fold, it evolved a core fold similar to that of well-characterized protein kinase-like domains, KxGQ motif completely occludes the typical substrate binding pocket.

Fig .1 A model of COQ8B 137-524 (Wheeler B., 2015)

Application of COQ8B Membrane Protein in Literature

Atmaca M., et al. Follow-up results of patients with ADCK4, mutations and the efficacy of CoQ10 treatment. Pediatric Nephrology. 2017, 32(8): 1369-1375. PubMed ID: 25449265

This article reports that COQ8B mutations are one of the most common causes of adolescent-onset albuminuria and/or CKD of unknown etiology in Turkey. CoQ10 supplementation appears efficacious at reducing proteinuria and may thereby be.
Korkmaz E, et al. ADCK4-Associated Glomerulopathy Causes Adolescence-Onset FSGS. Journal of the American Society of Nephrology Jasn. 2015, 27(1): 63. PubMed ID: 25967120

This article reveals that COQ8B-related glomerulopathy is an important novel differential diagnosis in adolescents with SRNS/FSGS and/or CKD of unknown origin.
Park E., et al. Focal segmental glomerulosclerosis and medullary nephrocalcinosis in children with ADCK4, mutations. Pediatric Nephrology. 2017, 32(9): 1-8. PubMed ID: 28405841

Authors in this group consider that early diagnosis of COQ8B mutations is essential because the condition is treatable with CoQ10 supplementation at an early stage. The association with medullary nephrocalcinosis may be an additional diagnostic indicator.
Vazquez L.F., et al. Mutations in COQ8B (ADCK4) found in patients with steroid resistant nephrotic syndrome alter COQ8B function. Human Mutation. 2017, 39(3). PubMed ID: 29194833

This article shows that COQ8B is a mitochondrial matrix protein peripherally associated with the inner membrane. Mutations in COQ8B carried by patients with steroid resistant nephrotic syndrome alter COQ8B function.
Ashraf S., et al. ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption. Journal of Clinical Investigation. 2013, 123(12): 5179-5189. PubMed ID: 24270420

Dates from this article indicate that individuals with SRNS with mutations in COQ8B or other genes that participate in CoQ10 biosynthesis may be treatable with CoQ10.

COQ8B Preparation Options

To obtain the soluble and functional target protein, the versatile Magic™ membrane protein production platform in Creative Biolabs enables many flexible options, from which you can always find a better match for your particular project. Aided by our versatile Magic™ anti-membrane protein antibody discovery platform, we also provide customized anti-COQ8B antibody development services.

As a forward-looking research institute as well as a leading custom service provider in the field of membrane protein, Creative Biolabs has won a good reputation among our worldwide customers for successfully accomplishing numerous challenging projects including generation of many functional membrane proteins. Please feel free to contact us for more information.

Reference

Wheeler B. Functional Characterization of Human ADCK3 and ADCK4, Mitochondrial Atypical Kinases. 2015, qspace.library.queensu.ca.

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