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Mouse Anti-PAPSS2 Recombinant Antibody (2A8) (CBMAB-P0763-YC)

Provided herein is a Mouse monoclonal antibody against Human 3'-phosphoadenosine 5'-phosphosulfate synthase 2. The antibody can be used for immunoassay techniques, such as ELISA, IF, IHC-P, WB.
See all PAPSS2 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
2A8
Antibody Isotype
IgG1, κ
Application
ELISA, IF, IHC-P, WB

Basic Information

Immunogen
PAPSS2 (NP_004661, 513-613 aa) partial recombinant protein with GST tag
Specificity
Human
Antibody Isotype
IgG1, κ
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!]

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

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

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