Entry - *182180 - SRP RECEPTOR SUBUNIT, ALPHA; SRPRA - OMIM
 
* 182180

SRP RECEPTOR SUBUNIT, ALPHA; SRPRA


Alternative titles; symbols

SIGNAL RECOGNITION PARTICLE RECEPTOR; SRPR
DOCKING PROTEIN; DP


HGNC Approved Gene Symbol: SRPRA

Cytogenetic location: 11q24.2   Genomic coordinates (GRCh38) : 11:126,235,930-126,268,895 (from NCBI)


TEXT

Description

The signal recognition particle receptor, also termed 'docking protein,' is an integral membrane protein of the endoplasmic reticulum (ER). Interaction of the SRPR with the signal recognition particle (SRP) on the arrested ribosome is essential for the protein targeting process in the ER membrane. SRP recognizes the signal sequences of nascent secretory proteins and arrests the elongation of the polypeptide chain, thus preventing completion of the presecretory protein. The SRPR releases the arrest and targets the nascent polypeptide chain to the membrane (summary by Yang-Feng et al., 1987).


Cloning and Expression

Hortsch et al. (1988) presented the complete nucleotide sequence of cDNA encoding the entire human docking protein and its deduced amino acid sequence.


Gene Function

Using protein crosslinking, Pool et al. (2002) detected distinct modes in the binding of SRP to the ribosome. During signal peptide recognition, SRP54 (604857) is positioned at the exit site close to ribosomal proteins L23a (602326) and L35 (611841). When SRP54 contacts the SRPR, SRP54 is rearranged such that it is no longer close to L23a. This repositioning may allow the translocon to dock with the ribosome, leading to insertion of the signal peptide into the translocation channel.


Mapping

Using a cDNA clone of dog kidney origin, Yang-Feng et al. (1987) mapped SRPR to 11q24-q25 by study of somatic cell hybrids and by in situ hybridization.

Janin et al. (1992) used PCR-based assay of somatic cell hybrids containing various fragments of 11q to map the SRPRA gene to the chromosomal region flanked by the 11q23 and 11q24 breakpoints associated with the constitutional and neuroepithelioma (11;22) translocations, respectively.


REFERENCES

  1. Hortsch, M., Labeit, S., Meyer, D. I. Complete cDNA sequence coding for human docking protein. Nucleic Acids Res. 16: 361-362, 1988. [PubMed: 3340536, related citations] [Full Text]

  2. Janin, N., Delattre, O., Lipinski, M. PCR-assisted localization of the human SRPR gene. Hum. Genet. 88: 583-585, 1992. [PubMed: 1312991, related citations] [Full Text]

  3. Pool, M. R., Stumm, J., Fulga, T. A., Sinning, I., Dobberstein, B. Distinct modes of signal recognition particle interaction with the ribosome. Science 297: 1345-1348, 2002. [PubMed: 12193787, related citations] [Full Text]

  4. Yang-Feng, T. L., Lauffer, L., Walter, P., Francke, U. Gene for the human signal recognition particle receptor (SRPR) mapped to chromosome 11q24-q25. (Abstract) Cytogenet. Cell Genet. 46: 723 only, 1987.


Contributors:
Ada Hamosh - updated : 10/18/2002
Creation Date:
Victor A. McKusick : 8/31/1987
carol : 03/31/2021
alopez : 03/28/2016
alopez : 5/8/2014
alopez : 10/23/2002
terry : 10/18/2002
carol : 6/3/1992
supermim : 3/16/1992
supermim : 3/20/1990
ddp : 10/27/1989
root : 6/20/1988
marie : 3/25/1988

* 182180

SRP RECEPTOR SUBUNIT, ALPHA; SRPRA


Alternative titles; symbols

SIGNAL RECOGNITION PARTICLE RECEPTOR; SRPR
DOCKING PROTEIN; DP


HGNC Approved Gene Symbol: SRPRA

Cytogenetic location: 11q24.2   Genomic coordinates (GRCh38) : 11:126,235,930-126,268,895 (from NCBI)


TEXT

Description

The signal recognition particle receptor, also termed 'docking protein,' is an integral membrane protein of the endoplasmic reticulum (ER). Interaction of the SRPR with the signal recognition particle (SRP) on the arrested ribosome is essential for the protein targeting process in the ER membrane. SRP recognizes the signal sequences of nascent secretory proteins and arrests the elongation of the polypeptide chain, thus preventing completion of the presecretory protein. The SRPR releases the arrest and targets the nascent polypeptide chain to the membrane (summary by Yang-Feng et al., 1987).


Cloning and Expression

Hortsch et al. (1988) presented the complete nucleotide sequence of cDNA encoding the entire human docking protein and its deduced amino acid sequence.


Gene Function

Using protein crosslinking, Pool et al. (2002) detected distinct modes in the binding of SRP to the ribosome. During signal peptide recognition, SRP54 (604857) is positioned at the exit site close to ribosomal proteins L23a (602326) and L35 (611841). When SRP54 contacts the SRPR, SRP54 is rearranged such that it is no longer close to L23a. This repositioning may allow the translocon to dock with the ribosome, leading to insertion of the signal peptide into the translocation channel.


Mapping

Using a cDNA clone of dog kidney origin, Yang-Feng et al. (1987) mapped SRPR to 11q24-q25 by study of somatic cell hybrids and by in situ hybridization.

Janin et al. (1992) used PCR-based assay of somatic cell hybrids containing various fragments of 11q to map the SRPRA gene to the chromosomal region flanked by the 11q23 and 11q24 breakpoints associated with the constitutional and neuroepithelioma (11;22) translocations, respectively.


REFERENCES

  1. Hortsch, M., Labeit, S., Meyer, D. I. Complete cDNA sequence coding for human docking protein. Nucleic Acids Res. 16: 361-362, 1988. [PubMed: 3340536] [Full Text: https://doi.org/10.1093/nar/16.1.361]

  2. Janin, N., Delattre, O., Lipinski, M. PCR-assisted localization of the human SRPR gene. Hum. Genet. 88: 583-585, 1992. [PubMed: 1312991] [Full Text: https://doi.org/10.1007/BF00219349]

  3. Pool, M. R., Stumm, J., Fulga, T. A., Sinning, I., Dobberstein, B. Distinct modes of signal recognition particle interaction with the ribosome. Science 297: 1345-1348, 2002. [PubMed: 12193787] [Full Text: https://doi.org/10.1126/science.1072366]

  4. Yang-Feng, T. L., Lauffer, L., Walter, P., Francke, U. Gene for the human signal recognition particle receptor (SRPR) mapped to chromosome 11q24-q25. (Abstract) Cytogenet. Cell Genet. 46: 723 only, 1987.


Contributors:
Ada Hamosh - updated : 10/18/2002

Creation Date:
Victor A. McKusick : 8/31/1987

Edit History:
carol : 03/31/2021
alopez : 03/28/2016
alopez : 5/8/2014
alopez : 10/23/2002
terry : 10/18/2002
carol : 6/3/1992
supermim : 3/16/1992
supermim : 3/20/1990
ddp : 10/27/1989
root : 6/20/1988
marie : 3/25/1988