Mouse Anti-KDSR (AA 1-333) Recombinant Antibody (CBXF-1007) (CBMAB-F0312-CQ)

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Datasheet

Summary

Host Animal

Mouse

Specificity

Human

Clone

CBXF-1007

Antibody Isotype

IgG1

Application

ELISA, WB

Basic Information

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

Liquid

Buffer

PBS, pH 7.2

Preservative

0.09% 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 1-333

Target

Full Name

3-ketodihydrosphingosine reductase

Introduction

The protein encoded by this gene catalyzes the reduction of 3-ketodihydrosphingosine to dihydrosphingosine. The putative active site residues of the encoded protein are found on the cytosolic side of the endoplasmic reticulum membrane. A chromosomal rearrangement involving this gene is a cause of follicular lymphoma, also known as type II chronic lymphatic leukemia. The mutation of a conserved residue in the bovine ortholog causes spinal muscular atrophy.

Entrez Gene ID

2531

UniProt ID

Q06136

Alternative Names

3-Ketodihydrosphingosine Reductase; 3-Dehydrosphinganine Reductase; Short Chain Dehydrogenase/Reductase Family 35C Member 1; Follicular Lymphoma Variant Translocation 1; Follicular Variant Translocation Protein 1; KDS Reductase; SDR35C1;

Function

Catalyzes the reduction of 3-ketodihydrosphingosine (KDS) to dihydrosphingosine (DHS).

Biological Process

3-keto-sphinganine metabolic processManual Assertion Based On ExperimentIDA:MGI
Sphingolipid biosynthetic processManual Assertion Based On ExperimentIBA:GO_Central

Cellular Location

Endoplasmic reticulum membrane

Involvement in disease

A chromosomal aberration involving KDSR is a cause of follicular lymphoma; also known as type II chronic lymphatic leukemia. Translocation t(2;18)(p11;q21) with a Ig J kappa chain region (PubMed:8417785).
Erythrokeratodermia variabilis et progressiva 4 (EKVP4):
A form of erythrokeratodermia variabilis et progressiva, a genodermatosis characterized by the coexistence of two independent skin lesions: transient erythema and hyperkeratosis that is usually localized but occasionally occurs in its generalized form. Clinical presentation varies significantly within a family and from one family to another. Palmoplantar keratoderma is present in around 50% of cases.

Topology

Cytoplasmic: 26-270
Helical: 271-291
Lumenal: 292-293
Helical: 294-314
Cytoplasmic: 315-332

More Infomation

References

Zhao, P., Zhuang, Z., Guan, X., Yang, J., Wang, W., & Kuang, Z. (2023). Crystal structure of the 3-ketodihydrosphingosine reductase TSC10 from Cryptococcus neoformans. Biochemical and Biophysical Research Communications, 670, 73-78.

Liu, Q., Chan, A. K., Chang, W. H., Yang, L., Pokharel, S. P., Miyashita, K., ... & Chen, C. W. (2022). 3-Ketodihydrosphingosine reductase maintains ER homeostasis and unfolded protein response in leukemia. Leukemia, 36(1), 100-110.

Pilz, R., Opálka, L., Majcher, A., Grimm, E., Van Maldergem, L., Mihalceanu, S., ... & Sandhoff, R. (2022). Formation of keto-type ceramides in palmoplantar keratoderma based on biallelic KDSR mutations in patients. Human Molecular Genetics, 31(7), 1105-1114.

Park, K. H., Ye, Z. W., Zhang, J., Hammad, S. M., Townsend, D. M., Rockey, D. C., & Kim, S. H. (2019). 3-ketodihydrosphingosine reductase mutation induces steatosis and hepatic injury in zebrafish. Scientific Reports, 9(1), 1138.

Bariana, T. K., Labarque, V., Heremans, J., Thys, C., De Reys, M., Greene, D., ... & Freson, K. (2019). Sphingolipid dysregulation due to lack of functional KDSR impairs proplatelet formation causing thrombocytopenia. Haematologica, 104(5), 1036.

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Protocols

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

Associated Products

Related Products

Mouse Anti-KDSR Recombinant Antibody (3E8)

Isotype control

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

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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Documents

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SDS
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