Mouse Anti-PAPSS2 Recombinant Antibody (2E7) (CBMAB-P0764-YC)

Name: Mouse Anti-PAPSS2 Recombinant Antibody (2E7)
SKU: CBMAB-P0764-YC
Rating: 5 (1 reviews)

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Datasheet

SDS

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

2E7

Application

IHC-P, WB

Specificity

Human

Antibody Isotype

IgG1

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

PBS, pH 7.3, 1% BSA, 50% glycerol, 0.02% sodium azide

Storage

Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.

Epitope

aa 1-247

More Infomation

Target

Full Name

PAPSS2

Introduction

Sulfation is a common modification of endogenous (lipids, proteins, and carbohydrates) and exogenous (xenobiotics and drugs) compounds. In mammals, the sulfate source is 3'-phosphoadenosine 5'-phosphosulfate (PAPS), created from ATP and inorganic sulfate. Two different tissue isoforms encoded by different genes synthesize PAPS. This gene encodes one of the two PAPS synthetases. Defects in this gene cause the Pakistani type of spondyloepimetaphyseal dysplasia. Two alternatively spliced transcript variants that encode different isoforms have been described for this gene.

Entrez Gene ID

9060

UniProt ID

O95340

Alternative Names

ATPSK2; BCYM4; SK2

Function

Bifunctional enzyme with both ATP sulfurylase and APS kinase activity, which mediates two steps in the sulfate activation pathway. The first step is the transfer of a sulfate group to ATP to yield adenosine 5'-phosphosulfate (APS), and the second step is the transfer of a phosphate group from ATP to APS yielding 3'-phosphoadenylylsulfate/PAPS, the activated sulfate donor used by sulfotransferases (PubMed:19474428, PubMed:11773860, PubMed:23824674, PubMed:25594860).
In mammals, PAPS is the sole source of sulfate while APS appears to only be an intermediate in the sulfate-activation pathway (PubMed:19474428, PubMed:11773860, PubMed:23824674, PubMed:25594860).
Plays indirectly an important role in skeletogenesis during postnatal growth (PubMed:9771708).

Biological Process

3'-phosphoadenosine 5'-phosphosulfate biosynthetic processManual Assertion Based On ExperimentIMP:UniProtKB
Blood coagulationIEA:Ensembl
Bone developmentIEA:Ensembl
Hormone metabolic processManual Assertion Based On ExperimentIMP:UniProtKB
Sulfate assimilationManual Assertion Based On ExperimentIMP:UniProtKB

Cellular Location

Cytosol

Involvement in disease

Brachyolmia type 4 with mild epiphyseal and metaphyseal changes (BCYM4):
A form of brachyolmia, a clinically and genetically heterogeneous skeletal dysplasia primarily affecting the spine and characterized by a short trunk, short stature, and platyspondyly. BCYM4 is an autosomal recessive form with mild epiphyseal and metaphyseal changes. Clinical features include short stature evidenced at birth, short and bowed lower limbs, mild brachydactyly, kyphoscoliosis, abnormal gait, enlarged knee joints. Some BCYM4 patients may manifest premature pubarche and hyperandrogenism associated with skeletal dysplasia and short stature.

Helvacıoğlu, D., & Güran, T. (2024). Bone Phenotype is Always Present But Androgen Excess is Less Frequently Seen in PAPSS2 Deficiency. Journal of Clinical Research in Pediatric Endocrinology, 16(1), 4.

Brylski, O., Shrestha, P., House, P. J., Gnutt, P., Mueller, J. W., & Ebbinghaus, S. (2022). Disease-related protein variants of the highly conserved enzyme PAPSS2 show marginal stability and aggregation in cells. Frontiers in Molecular Biosciences, 9, 860387.

Li, C., Wu, Y., Chen, B., Cai, Y., Guo, J., Leonard, A. S., ... & Wang, X. (2022). Markhor-derived introgression of a genomic region encompassing PAPSS2 confers high-altitude adaptability in Tibetan goats. Molecular Biology and Evolution, 39(12), msac253.

Mustafa, S., Hussain, M. F., Latif, M., Ijaz, M., Asif, M., Hassan, M., ... & Iqbal, F. (2022). A Missense Mutation (c. 1037 G> C, p. R346P) in PAPSS2 Gene Results in Autosomal Recessive form of Brachyolmia Type 1 (Hobaek Form) in A Consanguineous Family. Genes, 13(11), 2096.

Perez-Garcia, E. M., Whalen, P., & Gurtunca, N. (2022). Novel inactivating homozygous PAPSS2 mutation in two siblings with disproportionate short stature. AACE Clinical Case Reports, 8(2), 89-92.

Eltan, M., Yavas Abali, Z., Arslan Ates, E., Kirkgoz, T., Kaygusuz, S. B., Türkyılmaz, A., ... & Guran, T. (2020). Low DHEAS concentration in a girl presenting with short stature and premature pubarche: a novel PAPSS2 gene mutation. Hormone Research in Paediatrics, 92(4), 262-268.

Bownass, L., Abbs, S., Armstrong, R., Baujat, G., Behzadi, G., Berentsen, R. D., ... & Smithson, S. (2019). PAPSS2‐related brachyolmia: Clinical and radiological phenotype in 18 new cases. American Journal of Medical Genetics Part A, 179(9), 1884-1894.

Zhang, Y., Zou, X., Qian, W., Weng, X., Zhang, L., Zhang, L., ... & Hou, Z. (2019). Enhanced PAPSS2/VCAN sulfation axis is essential for Snail-mediated breast cancer cell migration and metastasis. Cell Death & Differentiation, 26(3), 565-579.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Antibody Pairs

PAPSS2 Matched Antibody Pair (841) (CAT#: APMAB-841LY)

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Isotype control

For research use only. Not intended for any clinical use.

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