S.NO	Uniprot ID	Protein Name	Sequence Length	RCC1 Repeats region	Function
1	Q15034	Probable E3 ubiquitin-protein ligase HERC3	1050	REPEAT 1 51 RCC1 1 REPEAT 52 101 RCC1 2 REPEAT 102 154 RCC1 3 REPEAT 156 207 RCC1 4 REPEAT 208 259 RCC1 5 REPEAT 261 311 RCC1 6 REPEAT 313 366 RCC1 7	E3 ubiquitin-protein ligase which accepts ubiquitin from an E2 ubiquitin-conjugating enzyme in the form of a thioester and then directly transfers the ubiquitin to targeted substrates
2	Q5GLZ8	Probable E3 ubiquitin-protein ligase HERC4	1057	REPEAT 1 51 RCC1 1 REPEAT 52 101 RCC1 2 REPEAT 102 154 RCC1 3 REPEAT 156 207 RCC1 4 REPEAT 208 259 RCC1 5 REPEAT 261 311 RCC1 6 REPEAT 313 368 RCC1 7	"Probable E3 ubiquitin-protein ligase involved in either protein trafficking or in the distribution of cellular structures Required for spermatozoon maturation and fertility, and for the removal of the cytoplasmic droplet of the spermatozoon E3 ubiquitin-protein ligases accept ubiquitin from an E2 ubiquitin-conjugating enzyme in the form of a thioester and then directly transfer it to targeted substrates"
3	Q9P258	Protein RCC2	522	REPEAT 103 165 RCC1 1 REPEAT 168 219 RCC1 2 REPEAT 221 271 RCC1 3 REPEAT 273 347 RCC1 4 REPEAT 348 401 RCC1 5 REPEAT 403 447 RCC1 6 REPEAT 448 501 RCC1 7	Required for completion of mitosis and cytokinesis May function as a guanine nucleotide exchange factor for the small GTPase RAC1
4	Q9UGK8	Secretion-regulating guanine nucleotide exchange factor	458	REPEAT 15 67 RCC1 1 REPEAT 68 119 RCC1 2 REPEAT 120 171 RCC1 3 REPEAT 172 230 RCC1 4 REPEAT 231 283 RCC1 5 REPEAT 284 351 RCC1 6 REPEAT 352 402 RCC1 7	"Probable guanine nucleotide exchange factor (GEF), which may be involved in the secretion process"
5	Q86SG6	Serine/threonine-protein kinase Nek8	692	REPEAT 312 350 RCC1 1 REPEAT 410 461 RCC1 2 REPEAT 462 513 RCC1 3 REPEAT 580 631 RCC1 4 REPEAT 632 684 RCC1 5	Required for renal tubular integrity May regulate local cytoskeletal structure in kidney tubule epithelial cells May regulate ciliary biogenesis through targeting of proteins to the cilia (By similarity) Plays a role in organogenesis and is involved in the regulation of the Hippo signaling pathway
6	P18754	Regulator of chromosome condensation	421	REPEAT 34 84 RCC1 1 REPEAT 85 136 RCC1 2 REPEAT 138 189 RCC1 3 REPEAT 191 257 RCC1 4 REPEAT 258 311 RCC1 5 REPEAT 312 362 RCC1 6 REPEAT 363 416 RCC1 7	"Guanine-nucleotide releasing factor that promotes the exchange of Ran-bound GDP by GTP, and thereby plays an important role in RAN-mediated functions in nuclear import and mitosis (PubMed:1944575, PubMed:17435751, PubMed:20668449, PubMed:22215983, PubMed:11336674) Contributes to the generation of high levels of chromosome-associated, GTP-bound RAN, which is important for mitotic spindle assembly and normal progress through mitosis (PubMed:12194828, PubMed:17435751, PubMed:22215983) Via its role in maintaining high levels of GTP-bound RAN in the nucleus, contributes to the release of cargo proteins from importins after nuclear import (PubMed:22215983) Involved in the regulation of onset of chromosome condensation in the S phase (PubMed:3678831) Binds both to the nucleosomes and double-stranded DNA (PubMed:17435751, PubMed:18762580)"
7	Q15751	Probable E3 ubiquitin-protein ligase HERC1	4861	REPEAT 371 420 RCC1 1 REPEAT 421 475 RCC1 2 REPEAT 476 528 RCC1 3 REPEAT 529 578 RCC1 4 REPEAT 580 631 RCC1 5 REPEAT 633 682 RCC1 6 REPEAT 683 735 RCC1 7 REPEAT 3996 4044 RCC1 8 REPEAT 4046 4099 RCC1 9 REPEAT 4101 4151 RCC1 10 REPEAT 4153 4203 RCC1 11 REPEAT 4205 4256 RCC1 12 REPEAT 4258 4308 RCC1 13 REPEAT 4310 4360 RCC1 14	"Involved in membrane trafficking via some guanine nucleotide exchange factor (GEF) activity and its ability to bind clathrin Acts as a GEF for Arf and Rab, by exchanging bound GDP for free GTP Binds phosphatidylinositol 4,5-bisphosphate, which is required for GEF activity May also act as a E3 ubiquitin-protein ligase which accepts ubiquitin from an E2 ubiquitin-conjugating enzyme in the form of a thioester and then directly transfers the ubiquitin to targeted substrates"
8	Q8TD19	Serine/threonine-protein kinase Nek9	979	REPEAT 388 444 RCC1 1 REPEAT 445 498 RCC1 2 REPEAT 499 550 RCC1 3 REPEAT 551 615 RCC1 4 REPEAT 616 668 RCC1 5 REPEAT 669 726 RCC1 6	"Pleiotropic regulator of mitotic progression, participating in the control of spindle dynamics and chromosome separation Phosphorylates different histones, myelin basic protein, beta-casein, and BICD2 Phosphorylates histone H3 on serine and threonine residues and beta-casein on serine residues Important for G1/S transition and S phase progression Phosphorylates NEK6 and NEK7 and stimulates their activity by releasing the autoinhibitory functions of Tyr-108 and Tyr-97 respectively"
9	Q8NDN9	RCC1 and BTB domain-containing protein 1	531	REPEAT 40 91 RCC1 1 REPEAT 93 145 RCC1 2 REPEAT 147 198 RCC1 3 REPEAT 199 250 RCC1 4 REPEAT 252 302 RCC1 5 REPEAT 304 356 RCC1 6	May be involved in cell cycle regulation by chromatin remodeling
10	Q8IVU3	Probable E3 ubiquitin-protein ligase HERC6	1022	REPEAT 41 92 RCC1 1 REPEAT 93 145 RCC1 2 REPEAT 147 198 RCC1 3 REPEAT 200 253 RCC1 4 REPEAT 254 304 RCC1 5	E3 ubiquitin-protein ligase which accepts ubiquitin from an E2 ubiquitin-conjugating enzyme in the form of a thioester and then directly transfers the ubiquitin to targeted substrates
11	O95714	E3 ubiquitin-protein ligase HERC2	4834	REPEAT 415 461 RCC1 1-1 REPEAT 462 512 RCC1 1-2 REPEAT 513 568 RCC1 1-3 REPEAT 569 620 RCC1 1-4 REPEAT 623 674 RCC1 1-5 REPEAT 675 726 RCC1 1-6 REPEAT 728 778 RCC1 1-7 REPEAT 2958 3009 RCC1 2-1 REPEAT 3010 3064 RCC1 2-2 REPEAT 3065 3116 RCC1 2-3 REPEAT 3118 3168 RCC1 2-4 REPEAT 3171 3222 RCC1 2-5 REPEAT 3224 3274 RCC1 2-6 REPEAT 3275 3326 RCC1 2-7 REPEAT 3951 4002 RCC1 3-1 REPEAT 4004 4056 RCC1 3-2 REPEAT 4058 4108 RCC1 3-3 REPEAT 4110 4162 RCC1 3-4 REPEAT 4164 4214 RCC1 3-5 REPEAT 4216 4266 RCC1 3-6 REPEAT 4268 4318 RCC1 3-7	"E3 ubiquitin-protein ligase that regulates ubiquitin-dependent retention of repair proteins on damaged chromosomes Recruited to sites of DNA damage in response to ionizing radiation (IR) and facilitates the assembly of UBE2N and RNF8 promoting DNA damage-induced formation of 'Lys-63'-linked ubiquitin chains Acts as a mediator of binding specificity between UBE2N and RNF8 Involved in the maintenance of RNF168 levels E3 ubiquitin-protein ligase that promotes the ubiquitination and proteasomal degradation of XPA which influences the circadian oscillation of DNA excision repair activity By controlling the steady-state expression of the IGF1R receptor, indirectly regulates the insulin-like growth factor receptor signaling pathway (PubMed:26692333)"
12	Q92834	X-linked retinitis pigmentosa GTPase regulator	1020	REPEAT 54 105 RCC1 1 REPEAT 106 158 RCC1 2 REPEAT 159 208 RCC1 3 REPEAT 209 261 RCC1 4 REPEAT 262 313 RCC1 5 REPEAT 314 367 RCC1 6	Could be a guanine-nucleotide releasing factor Plays a role in ciliogenesis Probably regulates cilia formation by regulating actin stress filaments and cell contractility Plays an important role in photoreceptor integrity May play a critical role in spermatogenesis and in intraflagellar transport processes (By similarity) May be involved in microtubule organization and regulation of transport in primary cilia
13	Q96I51	RCC1-like G exchanging factor-like protein	464	REPEAT 58 124 RCC1 1 REPEAT 128 191 RCC1 2 REPEAT 193 247 RCC1 3 REPEAT 248 300 RCC1 4 REPEAT 302 353 RCC1 5 REPEAT 354 411 RCC1 6 REPEAT 412 461 RCC1 7	"Guanine nucleotide exchange factor (GEF) for mitochondrial dynamin-related GTPase OPA1 Activates OPA1, by exchanging bound GDP for free GTP, and drives OPA1 and MFN1-dependent mitochondrial fusion (PubMed:28746876) Plays an essential role in mitochondrial ribosome biogenesis As a component of a functional protein-RNA module, consisting of RCC1L, NGRN, RPUSD3, RPUSD4, TRUB2, FASTKD2 and 16S mitochondrial ribosomal RNA (16S mt-rRNA), controls 16S mt-rRNA abundance and is required for intra-mitochondrial translation of core subunits of the oxidative phosphorylation system (PubMed:27667664)"
14	Q96Q42	Alsin	1657	REPEAT 59 108 RCC1 1 REPEAT 109 167 RCC1 2 REPEAT 169 218 RCC1 3 REPEAT 525 576 RCC1 4 REPEAT 578 627 RCC1 5	May act as a GTPase regulator Controls survival and growth of spinal motoneurons (By similarity)
15	A6NED2	RCC1 domain-containing protein 1	376	REPEAT 6 56 RCC1 1 REPEAT 176 227 RCC1 2 REPEAT 229 317 RCC1 3 REPEAT 318 371 RCC1 4	"Acts as a coregulator of KDM8 to promote histone demethylase activity on di- and trimethylated 'Lys-36' (H3K36me2/me3) of histone H3 (PubMed:24981860) Plays a role in transcriptional repression of satellite repeats, possibly by regulating H3K36 methylation levels in centromeric regions together with KDM8 (PubMed:24981860) Possibly together with KDM8, involved in proper mitotic spindle organization and chromosome segregation (PubMed:24981860) Plays a role in regulating alpha-tubulin deacetylation and cytoskeletal microtubule stability and thereby promoting cell migration and TGF-beta-induced epithelial to mesenchymal transition (EMT), potentially through the inhibition of KDM8 (PubMed:28455245)"
16	O75592	E3 ubiquitin-protein ligase MYCBP2	4678	REPEAT 600 655 RCC1 1 REPEAT 699 755 RCC1 2 REPEAT 907 957 RCC1 3 REPEAT 958 1008 RCC1 4 REPEAT 1010 1066 RCC1 5	"Atypical E3 ubiquitin-protein ligase which specifically mediates ubiquitination of threonine and serine residues on target proteins, instead of ubiquitinating lysine residues (PubMed:29643511) Shows esterification activity towards both threonine and serine, with a preference for threonine, and acts via two essential catalytic cysteine residues that relay ubiquitin to its substrate via thioester intermediates (PubMed:29643511) Interacts with the E2 enzymes UBE2D1, UBE2D3, UBE2E1 and UBE2L3 (PubMed:18308511, PubMed:29643511) Plays a key role in neural development, probably by mediating ubiquitination of threonine residues on target proteins (Probable) Involved in different processes such as regulation of neurite outgrowth, synaptic growth, synaptogenesis and axon degeneration (By similarity) Required for the formation of major central nervous system axon tracts (By similarity) Required for proper axon growth by regulating axon navigation and axon branching: acts by regulating the subcellular location and stability of MAP3K12/DLK (By similarity) Required for proper localization of retinogeniculate projections but not for eye-specific segregation (By similarity) Regulates axon guidance in the olfactory system (By similarity) Involved in Wallerian axon degeneration, an evolutionarily conserved process that drives the loss of damaged axons: acts by promoting destabilization of NMNAT2, probably via ubiquitination of NMNAT2 (By similarity) Catalyzes ubiquitination of threonine and/or serine residues on NMNAT2, consequences of threonine and/or serine ubiquitination are however unknown (PubMed:29643511) Regulates the internalization of TRPV1 in peripheral sensory neurons (By similarity) May mediate ubiquitination and subsequent proteasomal degradation of TSC2/tuberin (PubMed:18308511) Independently of the E3 ubiquitin-protein ligase activity, also acts as a guanosine exchange factor (GEF) for RAN in neurons of dorsal root ganglia (PubMed:26304119) May function as a facilitator or regulator of transcriptional activation by MYC (PubMed:9689053)"
17	Q9UII4	E3 ISG15--protein ligase HERC5	1024	REPEAT 96 155 RCC1 1 REPEAT 156 208 RCC1 2 REPEAT 209 260 RCC1 3 REPEAT 262 312 RCC1 4 REPEAT 314 364 RCC1 5	"Major E3 ligase for ISG15 conjugation Acts as a positive regulator of innate antiviral response in cells induced by interferon Functions as part of the ISGylation machinery that recognizes target proteins in a broad and relatively non-specific manner Catalyzes ISGylation of IRF3 which results in sustained activation, it attenuates IRF3-PIN1 interaction, which antagonizes IRF3 ubiquitination and degradation, and boosts the antiviral response Catalyzes ISGylation of influenza A viral NS1 which attenuates virulence; ISGylated NS1 fails to form homodimers and thus to interact with its RNA targets Catalyzes ISGylation of papillomavirus type 16 L1 protein which results in dominant-negative effect on virus infectivity Physically associated with polyribosomes, broadly modifies newly synthesized proteins in a cotranslational manner In an interferon-stimulated cell, newly translated viral proteins are primary targets of ISG15"
18	O75426	F-box only protein 24	580	REPEAT 376 425 RCC1	Substrate-recognition component of the SCF (SKP1-CUL1-F-box protein)-type E3 ubiquitin ligase complex

The 18 RCC1 Repeats proteins and their functions