Rabbit Polyclonal LEPREL1 antibody. Suitable for WB, IHC-P and reacts with Mouse, Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human P3H2 aa 300-550.
pH: 7.4
Preservative: 0.03% Proclin 300
Constituents: PBS, 50% Glycerol (glycerin, glycerine)
WB | IHC-P | |
---|---|---|
Human | Expected | Tested |
Mouse | Tested | Expected |
Species | Dilution info | Notes |
---|---|---|
Species Mouse | Dilution info 1/2000 - 1/5000 | Notes - |
Species | Dilution info | Notes |
---|---|---|
Species Human | Dilution info Use at an assay dependent concentration. | Notes - |
Species | Dilution info | Notes |
---|---|---|
Species Human | Dilution info 1/20 - 1/200 | Notes - |
Species | Dilution info | Notes |
---|---|---|
Species Mouse | Dilution info Use at an assay dependent concentration. | Notes - |
Prolyl 3-hydroxylase that catalyzes the post-translational formation of 3-hydroxyproline on collagens (PubMed:18487197). Contributes to proline 3-hydroxylation of collagen COL4A1 and COL1A1 in tendons, the eye sclera and in the eye lens capsule (By similarity). Has high activity with the type IV collagen COL4A1, and lower activity with COL1A1 (PubMed:18487197). Catalyzes hydroxylation of the first Pro in Gly-Pro-Hyp sequences where Hyp is 4-hydroxyproline (PubMed:18487197). Has no activity on substrates that lack 4-hydroxyproline in the third position (PubMed:18487197).
LEPREL1, MLAT4, P3H2, Prolyl 3-hydroxylase 2, Leprecan-like protein 1, Myxoid liposarcoma-associated protein 4
Rabbit Polyclonal LEPREL1 antibody. Suitable for WB, IHC-P and reacts with Mouse, Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human P3H2 aa 300-550.
pH: 7.4
Preservative: 0.03% Proclin 300
Constituents: PBS, 50% Glycerol (glycerin, glycerine)
Purity >95%.
LEPREL1 also known as Prolyl 3-hydroxylase 2 (P3H2) or leprecan-like 1 is an enzyme that plays an important role in the hydroxylation of proline residues in collagen a fundamental process in collagen maturation. LEPREL1 weighs about 76 kDa and is a part of a family of enzymes involved in post-translational modifications. LEPREL1 expression predominantly occurs in the eye specifically in the retina but it can also be found in other tissues such as the heart and skeletal muscle.
LEPREL1 performs mandatory activities in collagen triple-helix formation. It serves as a component of the endoplasmic reticulum-resident enzyme complex collaborating with additional enzymes like Collagen Prolyl Hydroxylase. These complexes ensure the correct folding of collagen fibrils contributing significantly to the structural integrity and function of connective tissues. Collagen biosynthesis mediated by LEPREL1 is essential for maintaining tissue architecture and functionality across various organ systems.
Collagen biosynthesis and modification represents an important pathway involving LEPREL1. The ER-resident enzymes such as PLOD1 and PLOD2 work alongside LEPREL1 emphasizing its positioning within collagen biosynthesis pathways. Proper collagen hydroxylation ensures the strength and elasticity of mature collagens highlighting its relevance in tissue development and repair.
LEPREL1 associates strongly with early-onset glaucoma and connective tissue disorders like Ehlers-Danlos syndrome. Mutations in LEPREL1 can lead to disruptions in collagen maturation causing weaker structural proteins and contributing to disease manifestation. Additionally research shows its involvement with MYOC (myocilin) in glaucoma pathogenesis further linking collagen processing defects with ocular health issues.
We have tested this species and application combination and it works. It is covered by our product promise.
We have not tested this specific species and application combination in-house, but expect it will work. It is covered by our product promise.
This species and application combination has not been tested, but we predict it will work based on strong homology. However, this combination is not covered by our product promise.
We do not recommend this combination. It is not covered by our product promise.
We are dedicated to supporting your work with high quality reagents and we are here for you every step of the way should you need us.
In the unlikely event of one of our products not working as expected, you are covered by our product promise.
Full details and terms and conditions can be found here:
Terms & Conditions.
Paraffin-embedded human liver tissue stained for LEPREL1 with ab222823 at 1/100 dilution in immunohistochemical analysis.
Paraffin-embedded human lung tissue stained for LEPREL1 with ab222823 at 1/100 dilution in immunohistochemical analysis.
All lanes: Western blot - Anti-LEPREL1 antibody (ab222823) at 1/2000 dilution
Lane 1: Mouse lung lysate
Lane 2: Mouse kidney lysate
All lanes: Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 62 kDa, 81 kDa
Observed band size: 25 kDa, 61 kDa, 65 kDa, 81 kDa, 90 kDa
Please note: All products are 'FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC OR THERAPEUTIC PROCEDURES'.
For licensing inquiries, please contact partnerships@abcam.com