Anti -GPSM2 (G-protein-signaling Modulator 2, Mosaic Protein LGN, LGN)

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 1; NC_000001.10 (109417972..109473044). Location: 1p13.3

Polyclonal

Goat

Recognizes human GPSM2. Species sequence homology: canine.

Affinity Purified
Purified by immunoaffinity chromatography.

Supplied as a liquid in TBS, pH 7.3, 0.5% BSA, 0.02% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Small volumes of anti-GPSM2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Plays a key role in retinoic acid metabolism. Acts on retinoids, including all-trans-retinoic acid (RA) and its stereoisomer 9-cis-RA. Capable of both 4-hydroxylation and 18-hydroxylation. Responsible for generation of several hydroxylated forms of RA, including 4-OH-RA, 4-oxo-RA and 18-OH-RA.

Antibodies; Abs to G Proteins

ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)

Suitable for use in ELISA, Western Blot and Immunohistochemistry.
Dilution: ELISA: 1:64,000
Western Blot: 0.01-0.03ug/ml
Immunohistochemistry (Formalin-fixed, paraffin-embedded): 5ug/ml

76,662 Da[Similar Products]

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GPSM2_HUMAN

The protein encoded by this gene belongs to a family of proteins that modulate activation of G proteins, which transduce extracellular signals received by cell surface receptors into integrated cellular responses. The N-terminal half of this protein contains 10 copies of leu-gly-asn (LGN) repeat, and the C-terminal half contains 4 GoLoco motifs, which are involved in guanine nucleotide exchange. This protein may play a role in neuroblast division and in the development of normal hearing. Mutations in this gene are associated with autosomal recessive nonsyndromic deafness (DFNB82).[provided by RefSeq, Jan 2011]

Function: Plays an important role in spindle pole orientation. Interacts and contributes to the functional activity of G(i) alpha proteins. Acts to stabilize the apical complex during neuroblast divisions. Ref.8

Subunit structure: Interacts with LLGL2. Interacts with INSC/inscuteable and probably with F2RL2. Ref.8 Ref.9

Subcellular location: Cytoplasm. Cytoplasm ? cell cortex. Note: Localizes in the cytoplasm in the interphase and at cell periphery in the metaphase. Ref.8

Tissue specificity: Ubiquitously expressed.

Involvement in disease: Chudley-McCullough syndrome (CMCS) [MIM:604213]: An autosomal recessive neurologic disorder characterized by early-onset sensorineural deafness and specific brain anomalies on MRI, including hypoplasia of the corpus callosum, enlarged cysterna magna with mild focal cerebellar dysplasia, and nodular heterotopia. Some patients have hydrocephalus. Psychomotor development is normal.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.11 Ref.13

Miscellaneous: Dysfunction of LGN is associated with the phenotype of multiple micronuclei due to chromosomal mis-segregation and defect in cell division through mis-localization of mitotic splindle regulator protein NuMA.

Sequence similarities: Belongs to the GPSM family.Contains 4 GoLoco domains.Contains 8 TPR repeats.

Caution: It is uncertain whether Met-1 or Met-8 is the initiator.Mutations in GPSM2 have been identified in people with profound congenital non-syndromic deafness designated as DFNB82 (Ref.11). Subsequent brain imaging of these individuals has revealed frontal polymicrogyria, abnormal corpus callosum, and gray matter heterotopia, consistent with a diagnosis of Chudley-McCullough syndrome (Ref.13).

Sequence caution: The sequence AAB40385.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.The sequence AAH27732.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.

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