Entry - *300214 - PLEXIN B3; PLXNB3 - OMIM
 
* 300214

PLEXIN B3; PLXNB3


Alternative titles; symbols

PLEXIN 6; PLXN6


HGNC Approved Gene Symbol: PLXNB3

Cytogenetic location: Xq28     Genomic coordinates (GRCh38): X:153,764,249-153,779,341 (from NCBI)


TEXT

Cloning and Expression

Tamagnone et al. (1999) identified the cDNA sequence of a novel member of the plexin gene family, which they called plexin B3.


Gene Function

Artigiani et al. (2004) showed that mammalian Plxnb3 is a high-affinity semaphorin-5A (SEMA5A; 609297) receptor. Plxnb3 was activated by Sema5a, and the Sema5a-Plxnb3 complex triggered intracellular signaling through Met (164860), the receptor for hepatocyte growth factor (142409).


Mapping

Tamagnone et al. (1999) found the PLXNB3 coding sequence in the genomic sequence of the ABCD1 gene (300371) on Xq28.


Molecular Genetics

In a 3-year-old Japanese boy with X-linked ALD (300100), Matsumoto et al. (2005) identified a partial deletion (exons 3 to 10) of the ABCD1 gene with fusion to the neighboring PLXNB3 gene. Sequencing showed that the breakpoints were at nucleotide +1374 in intron 2 of the ABCD1 gene and nucleotide +915 in intron 2 of the PLXNB3 gene. The mother was confirmed as a carrier of the deletion.


REFERENCES

  1. Artigiani, S., Conrotto, P., Fazzari, P., Gilestro, G. F., Barberis, D., Giordano, S., Comoglio, P. M., Tamagnone, L. Plexin-B3 is a functional receptor for semaphorin 5A. EMBO Rep. 5: 710-714, 2004. [PubMed: 15218527, images, related citations] [Full Text]

  2. Matsumoto, T., Miyake, N., Watanabe, Y., Yamanaka, G., Oana, S., Ogiwara, M., Hoshika, A., Sasaki, N., Miyahara, H., Niikawa, N. X-linked adrenoleukodystrophy with partial deletion of ALD due to fusion with the neighbor gene, PLXNB3. Am. J. Med. Genet. 138A: 300-302, 2005. [PubMed: 16152637, related citations] [Full Text]

  3. Tamagnone, L., Artigiani, S., Chen, H., He, Z., Ming, G., Song, H., Chedotal, A., Winberg, M. L., Goodman, C. S., Poo, M., Tessier-Lavigne, M., Comoglio, P. M. Plexins are a large family of receptors for transmembrane, secreted, and GPI-anchored semaphorins in vertebrates. Cell 99: 71-80, 1999. Note: Erratum: Cell 104: following 320, 2001. [PubMed: 10520995, related citations] [Full Text]


Patricia A. Hartz - updated : 12/19/2005
Marla J. F. O'Neill - updated : 10/25/2005
Creation Date:
Stylianos E. Antonarakis : 11/3/1999
terry : 07/27/2012
wwang : 1/24/2006
wwang : 12/19/2005
wwang : 10/27/2005
terry : 10/25/2005
carol : 1/13/2003
mgross : 11/23/1999
mgross : 11/3/1999

* 300214

PLEXIN B3; PLXNB3


Alternative titles; symbols

PLEXIN 6; PLXN6


HGNC Approved Gene Symbol: PLXNB3

Cytogenetic location: Xq28     Genomic coordinates (GRCh38): X:153,764,249-153,779,341 (from NCBI)


TEXT

Cloning and Expression

Tamagnone et al. (1999) identified the cDNA sequence of a novel member of the plexin gene family, which they called plexin B3.


Gene Function

Artigiani et al. (2004) showed that mammalian Plxnb3 is a high-affinity semaphorin-5A (SEMA5A; 609297) receptor. Plxnb3 was activated by Sema5a, and the Sema5a-Plxnb3 complex triggered intracellular signaling through Met (164860), the receptor for hepatocyte growth factor (142409).


Mapping

Tamagnone et al. (1999) found the PLXNB3 coding sequence in the genomic sequence of the ABCD1 gene (300371) on Xq28.


Molecular Genetics

In a 3-year-old Japanese boy with X-linked ALD (300100), Matsumoto et al. (2005) identified a partial deletion (exons 3 to 10) of the ABCD1 gene with fusion to the neighboring PLXNB3 gene. Sequencing showed that the breakpoints were at nucleotide +1374 in intron 2 of the ABCD1 gene and nucleotide +915 in intron 2 of the PLXNB3 gene. The mother was confirmed as a carrier of the deletion.


REFERENCES

  1. Artigiani, S., Conrotto, P., Fazzari, P., Gilestro, G. F., Barberis, D., Giordano, S., Comoglio, P. M., Tamagnone, L. Plexin-B3 is a functional receptor for semaphorin 5A. EMBO Rep. 5: 710-714, 2004. [PubMed: 15218527] [Full Text: https://doi.org/10.1038/sj.embor.7400189]

  2. Matsumoto, T., Miyake, N., Watanabe, Y., Yamanaka, G., Oana, S., Ogiwara, M., Hoshika, A., Sasaki, N., Miyahara, H., Niikawa, N. X-linked adrenoleukodystrophy with partial deletion of ALD due to fusion with the neighbor gene, PLXNB3. Am. J. Med. Genet. 138A: 300-302, 2005. [PubMed: 16152637] [Full Text: https://doi.org/10.1002/ajmg.a.30951]

  3. Tamagnone, L., Artigiani, S., Chen, H., He, Z., Ming, G., Song, H., Chedotal, A., Winberg, M. L., Goodman, C. S., Poo, M., Tessier-Lavigne, M., Comoglio, P. M. Plexins are a large family of receptors for transmembrane, secreted, and GPI-anchored semaphorins in vertebrates. Cell 99: 71-80, 1999. Note: Erratum: Cell 104: following 320, 2001. [PubMed: 10520995] [Full Text: https://doi.org/10.1016/s0092-8674(00)80063-x]


Contributors:
Patricia A. Hartz - updated : 12/19/2005
Marla J. F. O'Neill - updated : 10/25/2005

Creation Date:
Stylianos E. Antonarakis : 11/3/1999

Edit History:
terry : 07/27/2012
wwang : 1/24/2006
wwang : 12/19/2005
wwang : 10/27/2005
terry : 10/25/2005
carol : 1/13/2003
mgross : 11/23/1999
mgross : 11/3/1999