CNGB1 Membrane Protein Introduction

Introduction of CNGB1

CNGB1 is encoded by the CNGB1 geneandis also known as Cyclic nucleotide-gated cation channel beta-1, Cyclic nucleotide-gated cation channel 4, CNG channel 4, CNG channel beta-1, Glutamic acid-rich protein (GARP). It belongs to the Cyclic nucleotide-gated (CNG) ion channels family, which is cation-selective, opened by intracellular cyclic nucleotides like cAMP and cGMP, and present in many different neurons and non-neuronal cells.

Function of CNGB1 Membrane Protein

CNGB1 is required for the formation and/or outer segment targeting of the native rod CNG channel. Because functional homomeric CNGA1 channels can be formed in heterologous expression systems, it would appear that improper targeting is the underlying pathology in native rods, as a result of which the CNGA1 protein is presumably rapidly degraded. This rapid degradation is suggested by the lack of CNGA1 in rod inner segments and cell bodies of CNGB1^−/− retinas. The mechanistic role of CNGB1 in properly targeting CNGA1 to the outer segment remains unclear. The primary sequence of CNGA1 in the cytosolic N and C termini contains several KKXX motifs known as endoplasmatic reticulum retention/retrieval motifs that mediate membrane trafficking for a host of proteins. Moreover, mice deficient in CNGB1 develop a retinal degeneration that is similar to human retinitis pigmentosa (RP), including a group of genetically diverse diseases that are characterized by progressive degeneration of rods and subsequently cones.

Fig.1 A simplified schematic diagram of the structure of CNGB1 channels (Barry, 2008).

Application of CNGB1 Membrane Protein in Literature

1. Hüttl S., et al. Impaired Channel Targeting and Retinal Degeneration in Mice Lacking the Cyclic Nucleotide-Gated Channel Subunit CNGB1. The Journal of neuroscience: the official journal of the Society for Neuroscience. 2005, 25(1):130-138. PubMed ID: 15634774
   
   

   This article reports that CNGB1 is a crucial determinant of native CNG channel targeting. As a result of the lack of rod CNG channels, CNGB1^−/− mice develop a retinal degeneration that resembles human retinitis pigmentosa.

2. Charbel I.P., et al. Olfactory dysfunction in patients with cngb1-associated retinitis pigmentosa. JAMA Ophthalmology. 2018, 136(7):761-769. PubMed ID: 29800053
   
   

   This article reveals that the mutations of CNGB1 are involved in the autosomal recessive RP-olfactory dysfunction syndrome, which is characterized by a slow progression of retinal degeneration and variable anosmia or hyposmia.

3. Blank T., et al. Early Microglia Activation Precedes Photoreceptor Degeneration in a Mouse Model of CNGB1-Linked Retinitis Pigmentosa. Frontiers in Immunology. 2017, 8:1930. PubMed ID: 29354133
   
   

   Authors in this group perform a combination of FACS analysis, immunohistochemistry and gene expression analysis to demonstrate that the CNGB1 may be responsible for the development of an early, pre-degenerative stage of microglia in the retina.

4. Hull S., et al. Clinical characterization of cngb1-related autosomal recessive retinitis pigmentosa. JAMA Ophthalmology. 2017, 135(2):137-144. PubMed ID: 28056120
   
   

   This article identifies a total of 8 different variants in CNGB1 and they are common in British white population. CNGB1 variants show the progressive RP phenotype in patients.

5. Winkler P.A., et al. A Large Animal Model for CNGB1 Autosomal Recessive Retinitis Pigmentosa. PLOS ONE. 2013, 8(8):e72229. PubMed ID: 23977260
   
   

   The authors in this article perform a population study to show that the CNGB1 mutation accounts for ~70% of cases of Papillon PRA in PRA-affected canine DNA bank, indicating that CNGB1 mutations are involved in the autosomal recessive RP.

CNGB1 Preparation Options

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Reference

1. Barry P H., et al. (2008) Biophysics of CNG Ion Channels. Sensing with Ion Channels, ed Martinac B (Springer Berlin Heidelberg, Berlin, Heidelberg), pp 175-200.

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