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

Name: Mouse Anti-KDSR (AA 1-333) Recombinant Antibody (CBXF-1007)
SKU: CBMAB-F0312-CQ
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

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

Basic Information

Host Animal

Mouse

Clone

CBXF-1007

Application

ELISA, 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

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

More Infomation

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

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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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)
Proteogenomic
Other Protocols

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Isotype control

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

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