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Mouse Anti-SPG7 Recombinant Antibody (1C1) (CBMAB-P3685-YC)

Provided herein is a Mouse monoclonal antibody against Human SPG7, Paraplegin Matrix AAA Peptidase Subunit. The antibody can be used for immunoassay techniques, such as FC, IF, WB.
See all SPG7 antibodies
Published Data
Fig.1 Western blot images representative of three independent experiments are shown.
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Fig.1 Loss of AFG3L2 and/or YME1L is associated with elevated OMA1, diminished SPG7, and a markedly altered pattern of OPA1 protein forms. Whole-cell lysates were resolved using western blotted with antibody to Drp1, Oma1, and Spg7.
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Fig.2 Immunohistochemistry showing tyrosine hydroxylase (TH)-positive cells in the substantia nigra also express paraplegin, a mitochondrial regulatory protein.
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Summary

Host Animal
Mouse
Specificity
Human
Clone
1C1
Antibody Isotype
IgG1
Application
FC, IF, 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
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 300-573

Target

Full Name
SPG7, Paraplegin Matrix AAA Peptidase Subunit
Introduction
SPG7 is a mitochondrial metalloprotease protein that is a member of the AAA family. Members of this protein family share an ATPase domain and have roles in diverse cellular processes including membrane trafficking, intracellular motility, organelle biogenesis, protein folding, and proteolysis. Mutations in this gene cause autosomal recessive spastic paraplegia 7.
Entrez Gene ID
6687
UniProt ID
Q9UQ90
Alternative Names
SPG7, Paraplegin Matrix AAA Peptidase Subunit; Cell Matrix Adhesion Regulator; Spastic Paraplegia 7 (Pure And Complicated Autosomal Recessive); Spastic Paraplegia 7 Protein; Paraplegin; CMAR;
Function
ATP-dependent zinc metalloprotease. Plays a role in the formation and regulation of the mitochondrial permeability transition pore (mPTP) and its proteolytic activity is dispensable for this function (PubMed:26387735).
Biological Process
Biological Process anterograde axonal transportIEA:Ensembl
Biological Process mitochondrial outer membrane permeabilization involved in programmed cell deathManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process mitochondrial protein processingManual Assertion Based On ExperimentIBA:GO_Central
Biological Process nervous system developmentManual Assertion Based On ExperimentTAS:ProtInc
Biological Process protein-containing complex assemblyManual Assertion Based On ExperimentIBA:GO_Central
Biological Process proteolysisManual Assertion Based On ExperimentTAS:ProtInc
Biological Process regulation of mitochondrial membrane permeabilityManual Assertion Based On ExperimentIMP:UniProtKB
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Spastic paraplegia 7, autosomal recessive (SPG7):
A form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. SPG7 is a complex form. Additional clinical features are cerebellar syndrome, supranuclear palsy, and cognitive impairment, particularly disturbance of attention and executive functions.
Topology
Mitochondrial matrix: 106-144
Helical: 145-165
Mitochondrial intermembrane: 166-248
Helical: 249-269
Mitochondrial matrix: 270-795
PTM
Upon import into the mitochondrion, the N-terminal transit peptide is cleaved by the mitochondrial-processing peptidase (MPP) to generate an intermediate form which undergoes a second proteolytic cleavage mediated by proteases AFG3L1 and/or AFG3L2 removing an additional N-terminal fragment to generate the proteolytically active mature form.
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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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